[Federal Register Volume 73, Number 53 (Tuesday, March 18, 2008)]
[Rules and Regulations]
[Pages 14570-14616]
From the Federal Register Online via the Government Publishing Office [www.gpo.gov]
[FR Doc No: E8-5023]



[[Page 14569]]

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Part II





Social Security Administration





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20 CFR Part 404



Revised Medical Criteria for Evaluating Immune System Disorders; Final 
rule

  Federal Register / Vol. 73, No. 53 / Tuesday, March 18, 2008 / Rules 
and Regulations  

[[Page 14570]]


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SOCIAL SECURITY ADMINISTRATION

20 CFR Part 404

[Docket No. SSA 2006-0070]
RIN 0960-AF33


Revised Medical Criteria for Evaluating Immune System Disorders

AGENCY: Social Security Administration.

ACTION: Final Rules.

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SUMMARY: We are revising the criteria in the Listing of Impairments 
(the listings) that we use to evaluate claims involving immune system 
disorders. We apply these criteria when you claim benefits based on 
disability under title II and title XVI of the Social Security Act (the 
Act). The revisions reflect our adjudicative experience, as well as 
advances in medical knowledge, treatment, and methods of evaluating 
immune system disorders.

DATES: These rules are effective June 16, 2008.

FOR FURTHER INFORMATION CONTACT: Paul Scott, Office of Compassionate 
Allowances and Listings Improvement, Social Security Administration, 
4422 Annex Building, 6401 Security Boulevard, Baltimore, Maryland 
21235-6401, (410) 966-1192. For information on eligibility or filing 
for benefits, call our national toll-free number, 1-800-772-1213 or TTY 
1-800-325-0778, or visit our Internet Web site, Social Security Online 
at http://www.socialsecurity.gov/.

SUPPLEMENTARY INFORMATION: 

Electronic Version

    The electronic file of this document is available on the date of 
publication in the Federal Register at http://www.gpoaccess.gov/fr/index.html.

Background

    We are revising and making final the rules we proposed for 
evaluating immune system disorders in the Notice of Proposed Rulemaking 
(NPRM) published in the Federal Register on August 4, 2006 (71 FR 
44432, corrected at 71 FR 46983). We provide a summary of the 
provisions of the final rules below, with an explanation of the changes 
we have made from the text in the NPRM. We then provide summaries of 
the public comments on the NPRM and our reasons for adopting or not 
adopting the recommendations in those comments in the section ``Public 
Comments on the NPRM.'' The final rule language follows that section.

What Programs Do These Final Rules Affect?

    These final rules affect disability determinations and decisions 
that we make under title II and title XVI of the Act. In addition, to 
the extent that Medicare entitlement and Medicaid eligibility are based 
on whether you qualify for disability benefits under title II and title 
XVI, these final rules also affect the Medicare and Medicaid programs.

Who Can Get Disability Benefits?

    Under title II of the Act, we provide for the payment of disability 
benefits if you are disabled and belong to one of the following three 
groups:
     Workers insured under the Act,
     Children of insured workers, and
     Widows, widowers, and surviving divorced spouses (see 
Sec.  404.336) of insured workers.
    Under title XVI of the Act, we provide for Supplemental Security 
Income (SSI) payments on the basis of disability if you are disabled 
and have limited income and resources.

How do we define disability?

    Under both the title II and title XVI programs, disability must be 
the result of any medically determinable physical or mental impairment 
or combination of impairments that is expected to result in death or 
which has lasted or is expected to last for a continuous period of at 
least 12 months. Our definitions of disability are shown in the 
following table:

------------------------------------------------------------------------
                                                    Disability means you
                                                      have a medically
 If you file a claim under . .  And you are . . .       determinable
               .                                      impairment(s) as
                                                    described above that
                                                      results in . . .
------------------------------------------------------------------------
title II......................  an adult or a      the inability to do
                                 child.             any substantial
                                                    gainful activity
                                                    (SGA).
title XVI.....................  an individual age  the inability to do
                                 18 or older.       any SGA.
title XVI.....................  an individual      marked and severe
                                 under age 18.      functional
                                                    limitations.
------------------------------------------------------------------------

How do we decide whether you are disabled?

    If you are applying for benefits under title II of the Act, or if 
you are an adult applying for payments under title XVI of the Act, we 
use a five-step ``sequential evaluation process'' to decide whether you 
are disabled. We describe this five-step process in our regulations at 
Sec. Sec.  404.1520 and 416.920. We follow the five steps in order and 
stop as soon as we can make a determination or decision. The steps are:
    1. Are you working, and is the work you are doing substantial 
gainful activity? If you are working and the work you are doing is 
substantial gainful activity, we will find that you are not disabled, 
regardless of your medical condition or your age, education, and work 
experience. If you are not, we will go on to step 2.
    2. Do you have a ``severe'' impairment? If you do not have an 
impairment or combination of impairments that significantly limits your 
physical or mental ability to do basic work activities, we will find 
that you are not disabled. If you do, we will go on to step 3.
    3. Do you have an impairment(s) that meets or medically equals the 
severity of an impairment in the listings? If you do, and the 
impairment(s) meets the duration requirement, we will find that you are 
disabled. If you do not, we will go on to step 4.
    4. Do you have the residual functional capacity (RFC) to do your 
past relevant work? If you do, we will find that you are not disabled. 
If you do not, we will go on to step 5.
    5. Does your impairment(s) prevent you from doing any other work 
that exists in significant numbers in the national economy, considering 
your RFC, age, education, and work experience? If it does, and it meets 
the duration requirement, we will find that you are disabled. If it 
does not, we will find that you are not disabled.
    We use a different sequential evaluation process for children who 
apply for payments based on disability under title XVI of the Act. We 
describe that sequential evaluation process in Sec.  416.924 of our 
regulations. If you are already receiving benefits, we also use a 
different sequential evaluation process when we decide whether your 
disability continues. See Sec. Sec.  404.1594, 416.994, and 416.994a of 
our regulations. However, all of the processes include steps at which 
we consider whether your impairment(s) meets or medically equals one of 
our listings.

What are the listings?

    The listings are examples of impairments that we consider severe 
enough to prevent you as an adult from doing any gainful activity. If 
you are a child seeking SSI payments based on disability, the listings 
describe

[[Page 14571]]

impairments that we consider severe enough to result in marked and 
severe functional limitations. Although the listings are contained only 
in appendix 1 to subpart P of part 404 of our regulations, we 
incorporate them by reference in the SSI program in Sec.  416.925 of 
our regulations and apply them to claims under both title II and title 
XVI of the Act.

How do we use the listings?

    The listings are in two parts. There are listings for adults (part 
A) and for children (part B). If you are an individual age 18 or over, 
we apply the listings in part A when we assess your claim, and we never 
use the listings in part B.
    If you are an individual under age 18, we first use the criteria in 
part B of the listings. Part B contains criteria that apply only to 
individuals who are under age 18. If the criteria in part B do not 
apply, we may use the criteria in part A when those criteria give 
appropriate consideration to the effects of the impairment(s) in 
children. (See Sec. Sec.  404.1525 and 416.925.)
    If your impairment(s) does not meet any listing, we will also 
consider whether it medically equals any listing; that is, whether it 
is as medically severe as an impairment in the listings. (See 
Sec. Sec.  404.1526 and 416.926.)

What if you do not have an impairment(s) that meets or medically equals 
a listing?

    We use the listings only to decide that you are disabled or that 
you are still disabled. We will not deny your claim or decide that you 
no longer qualify for benefits because your impairment(s) does not meet 
or medically equal a listing. If you have a severe impairment(s) that 
does not meet or medically equal any listing, we may still find you 
disabled based on other rules in the ``sequential evaluation process.'' 
Likewise, we will not decide that your disability has ended only 
because your impairment(s) no longer meets or medically equals a 
listing.
    Also, when we conduct reviews to determine whether your disability 
continues, we will not find that your disability has ended because we 
have changed a listing. Our regulations explain that, when we change 
our listings, we continue to use our prior listings when we review your 
case, if you qualified for disability benefits or SSI payments based on 
our determination or decision that your impairment(s) met or medically 
equaled a listing. In these cases, we determine whether you have 
experienced medical improvement and, if so, whether the medical 
improvement is related to the ability to work. If your condition has 
medically improved so that you no longer meet or medically equal the 
prior listing, we evaluate your case further to determine whether you 
are currently disabled. We may find that you are currently disabled, 
depending on the full circumstances of your case. See Sec. Sec.  
404.1594(c)(3)(i) and 416.994(b)(2)(iv)(A). If you are a child who is 
eligible for SSI payments, we follow a similar rule when we decide that 
you have experienced medical improvement in your condition. See Sec.  
416.994a(b)(2).

Why are we revising the listings for immune system disorders?

    We are making these revisions to update the medical criteria in the 
listings and to provide more information about how we evaluate immune 
system disorders. We first published these rules in 1993 (58 FR 36008). 
At that time, we established body system listings for immune system 
disorders in part A and part B. We made those rules effective for 5 
years from the date of publication, unless we extended them, or revised 
and issued them again (58 FR at 36051). Since that time, we have 
extended the expiration date of the immune body system listings but we 
have not comprehensively revised them.
    We have, however, made several changes to these listings over the 
years. On November 19, 2001, we published final rules in the Federal 
Register adding listings 14.09 and 114.09, for inflammatory arthritis, 
to the immune system listings, and adding introductory text for those 
listings in sections 14.00B6 and 114.00E (66 FR 58009). We published 
minor technical changes to the immune system listings on February 24, 
2002 (67 FR 20018).

How did we develop these final rules?

    These final rules reflect our adjudicative experience and advances 
in medical knowledge, treatment, and methods of evaluating immune 
system disorders. They also reflect comments on the NPRM we published 
in 2006.
    Before we developed the NPRM, we published an Advance Notice of 
Proposed Rulemaking (ANPRM) in the Federal Register on May 9, 2003 (68 
FR 24896). The purpose of the ANPRM was to inform the public that we 
were planning to update and revise the rules we use to evaluate immune 
system disorders and to invite interested individuals and organizations 
to send us comments and suggestions for updating and revising the 
immune system listings. In the ANPRM, we provided a 60-day period for 
comments and suggestions; that period ended on July 8, 2003. We 
received over 200 letters and e-mails in response to the notice, many 
from individuals who have immune system disorders or who have family 
members with such disorders. We also received comments from medical 
experts, advocates, and people who adjudicate claims for us. Although 
we are not summarizing or responding to the ANPRM comments in these 
final rules, we read and considered them carefully.
    We also hosted policy conferences on ``Immune System Disorders in 
the Disability Programs'' in Philadelphia, PA, on December 15, 2003, 
and in San Francisco, CA, on February 18 and 19, 2004. At these 
conferences, we heard comments and suggestions for updating and 
revising these rules from individuals who have immune system disorders 
and their family members, physicians who treat individuals with immune 
system disorders, other professionals who work with people who have 
immune system disorders, advocates who represent individuals with 
immune system disorders, and individuals who make disability 
determinations and decisions for us in the State agencies and the 
Office of Disability Adjudication and Review (formerly called the 
Office of Hearings and Appeals).
    As already noted, these final rules also reflect comments we asked 
you to provide on the NPRM. We summarize and respond to those comments 
later in this preamble. Throughout this preamble, we refer to ``public 
comments on the NPRM'' whenever we refer to these comments to 
distinguish them from public comments we received on the ANPRM and at 
the outreach meetings.

What do we mean by ``final rules'' and ``prior rules''?

    Even though these rules will not go into effect until 90 days after 
publication of this notice, for clarity, we refer to the changes we are 
making here as the ``final rules'' and to the rules that will be 
changed by these final rules as the ``prior rules.''

When will we start to use these final rules?

    We will start to use these final rules on their effective date. We 
will continue to use our prior rules until the effective date of these 
final rules. When these final rules become effective, we will apply 
them to new applications filed on or after the effective date of these 
rules and to claims pending before us, as we describe below.

[[Page 14572]]

    As is our usual practice when we make changes to our regulations, 
we will apply these final rules on or after their effective date 
whenever we make a determination or decision, including in those claims 
in which we make a determination or decision after a remand to us from 
a Federal court. With respect to claims in which we have made a final 
decision and that are pending judicial review in Federal court, we 
expect that the court would review the Commissioner's final decision in 
accordance with the rules in effect at the time the final decision of 
the Commissioner was issued. If a court reverses the Commissioner's 
final decision and remands the case for further administrative 
proceedings after the effective date of these final rules, we will 
apply the provisions of these final rules to the entire period at issue 
in the claim in our new decision issued pursuant to the court's remand.

How long will these final rules be effective?

    These final rules will no longer be effective 8 years after the 
date on which they become effective, unless we extend them or revise 
and issue them again. However, we intend to monitor these rules, and if 
needed, will update the criteria for any impairment in these rules 
before the end of the 8-year period.

What revisions are we making with these final rules?

    We are revising the prior rules to:
     Expand, reorganize, and update the introductory text in 
final 14.00 and 114.00 to provide more guidance for our adjudicators, 
and to reflect the revised listings.
     Add paragraph headings to the introductory text in final 
14.00 and 114.00 for easier reference.
     Add final 14.00C and 114.00C to explain the meaning of key 
terms.
     Remove all reference listings. Reference listings are 
listings that are met by satisfying the criteria of another listing. 
For example, prior listing 14.08G1 for human immunodeficiency virus 
(HIV) infection with anemia was a reference listing that required 
evaluation under current listing 7.02 for chronic anemia. Therefore, 
prior listing 14.08G1 was redundant. In some cases, instead of using 
reference listings, we provide general guidance in the introductory 
text for the immune system disorders listings (final 14.00J2g) stating 
that impairments in other body systems that result from immune system 
disorders should be evaluated under the criteria of the affected body 
system. In other cases, we are replacing reference listings with 
specific listing criteria that are appropriate for evaluation under 
this body system. For example, prior listing 14.06, for 
undifferentiated connective tissue disorders, was entirely a reference 
listing. In the final rules, we are replacing the reference listing 
criterion with criteria that are specific to these disorders.
     Add final listings 14.10 and 114.10 for evaluating 
Sjogren's syndrome.
     Add functional criteria to the listings, similar to those 
in prior HIV infection listings 14.08N and 114.08O, for each of the 
other listed immune system disorders (for example, systemic lupus 
erythematosus and systemic vasculitis).
     Make nonsubstantive editorial changes to update the 
medical terminology in the introductory text and the listings and to 
make their language simpler and clearer.

How are we changing the introductory text for the immune system 
disorders listings for adults?

    We are expanding and reorganizing the introductory text for these 
listings. There were four major sections in prior 14.00, and the 
longest of those sections, 14.00D, addressed only the evaluation of HIV 
infection. In these final rules, we are adding more sections and 
expanding the guidance we provide about evaluating other kinds of 
immune system disorders.
    Some of the guidance in prior 14.00D was useful for evaluating 
other kinds of immune system disorders in addition to HIV infection. 
Therefore, we are moving that guidance from prior 14.00D to new 
sections that have more general applicability to immune system 
disorders. We are not removing any substantive guidance about how we 
evaluate HIV infection, only reorganizing some of the information that 
was in 14.00D of the prior rules and giving it broader applicability 
where appropriate. We are also updating and expanding some of the 
guidance for evaluating HIV infection and its effects that was in the 
prior rules, as we describe in more detail below.
    The four sections in the prior rules were:
     Prior 14.00A, a short paragraph that described generally 
the kinds of disorders we include in this body system.
     Prior 14.00B, a lengthy section that discussed the 
evaluation of connective tissue disorders; that is, autoimmune 
disorders. It included six undesignated paragraphs that primarily 
explained the kinds of evidence we need to document the existence and 
severity of these disorders, including how we evaluate loss of 
function. These paragraphs were followed by six numbered sections that 
provided guidance about specific impairments in the listings.
     Prior 14.00C, a single sentence that explained that we 
evaluate allergic disorders under the appropriate listing of the 
affected body system.
     Prior 14.00D, a lengthy section that explained how we 
documented the existence and severity of HIV infection, including how 
we evaluated loss of function under prior listing 14.08N. It included 
eight numbered subsections and many paragraphs that were not designated 
with letters or numbers within those subsections.
    In the final rules, there are 10 sections in the introductory text. 
The first three sections (final 14.00A, B, and C) provide general 
information about this body system, including definitions of terms. 
Each of the next three sections describes a particular category or type 
of immune system disorder: Autoimmune disorders (final 14.00D); immune 
deficiency disorders, excluding HIV infection (final 14.00E); and HIV 
infection (final 14.00F). The next three sections explain how we 
consider the effects of your treatment (final 14.00G), your symptoms 
(final 14.00H), and the functional limitations from your immune system 
disorder under these listings (final 14.00I). The last section, final 
14.00J, explains how we consider the effects of your immune system 
disorder when it does not meet the requirements of one of the immune 
system disorders listings. We are designating all paragraphs in the 
final rules with letters or numbers for easier reference. We are also 
providing headings for all of the major sections and many of the 
subsections.
    The following are the names of the major sections in final 14.00. 
We describe each section in detail later in this preamble.
     Final 14.00A: What disorders do we evaluate under the 
immune system disorders listings?
     Final 14.00B: What information do we need to show that you 
have an immune system disorder?
     Final 14.00C: Definitions
     Final 14.00D: How do we document and evaluate the listed 
autoimmune disorders?
     Final 14.00E: How do we document and evaluate immune 
deficiency disorders, excluding HIV infection?
     Final 14.00F: How do we document and evaluate human 
immunodeficiency virus (HIV) infection?
     Final 14.00G: How do we consider the effects of treatment 
in evaluating your autoimmune disorder, immune deficiency disorder, or 
HIV infection?

[[Page 14573]]

     Final 14.00H: How do we consider your symptoms, including 
your pain, severe fatigue, and malaise?
     Final 14.00I: How do we use the functional criteria in 
these listings?
     Final 14.00J: How do we evaluate your immune system 
disorder when it does not meet one of these listings?
    The following is a detailed description of the changes in the 
introductory text.

14.00 Immune System Disorders

    We are changing the name of this body system from ``Immune System'' 
to ``Immune System Disorders'' to more accurately reflect that we use 
these listings to evaluate immune system disorders in accordance with 
the requirements of the disability program.

Final 14.00A--What disorders do we evaluate under the immune system 
disorders listings?

    In final 14.00A, we provide a brief overview of this body system. 
We explain the kinds of disorders we evaluate under the immune system 
disorders listings and that we organize these impairments under the 
categories of ``autoimmune disorders,'' ``immune deficiency disorders, 
excluding HIV infection,'' and ``HIV infection.'' Final 14.00A has four 
subsections.
    We incorporate prior 14.00A in the opening sentence of final 
14.00A1. We are revising the sentence, which explains the kinds of 
immune system dysfunction that immune system disorders may cause, to 
update and simplify it. In final 14.00A1a and 14.00A1b, we incorporate 
the first sentence in the sixth paragraph of prior 14.00B to explain 
that immune system disorders can cause dysfunction in one or more 
components of the immune system, and describe ways in which immune 
system disorders may result in loss of function. In the third sentence 
of final 14.00A1b, we are adding ``involuntary'' as a descriptor of 
weight loss to clarify that we mean weight loss due to an immune system 
disorder(s) or its treatment. We are adding ``involuntary'' as a 
descriptor of weight loss throughout the introductory text in part A 
and part B for this same reason. Final 14.00A1c is a new paragraph that 
explains how we have organized the discussions of immune system 
disorders in the introductory text for these listings.
    In final 14.00A2, Autoimmune disorders, we incorporate the first 
paragraph in prior 14.00B to provide a brief description of autoimmune 
disorders. We are adding an explanation that these disorders are 
sometimes referred to as ``rheumatic diseases,'' ``connective tissue 
disorders,'' or ``collagen vascular disorders,'' and that some of the 
features of these disorders in adults differ from the features of the 
same disorders in children. We provide a cross-reference to final 
14.00D, the section of the introductory text that addresses autoimmune 
disorders in detail. We are also removing the last sentence of the 
first paragraph of prior 14.00B, which explained that connective tissue 
disorders generally evolve and persist over time, may result in 
functional loss, and may require long-term, repeated evaluation and 
management, because it did not provide useful adjudicative guidance. 
However, we do explain in final 14.00A1b that immune system disorders 
can cause ``extreme'' loss of function. We also explain parenthetically 
that ``extreme'' means ``very serious'' to make clear that we use the 
term ``extreme'' in the same way that we use it in other body systems; 
for example, see 1.00B2b1 and 1.00B2c in the musculoskeletal system.
    Final 14.00A3, Immune deficiency disorders, excluding HIV 
infection, is new. We explain that these disorders can be classified as 
``primary'' or ``acquired,'' are characterized by recurrent or unusual 
infections, and are associated with an increased risk of malignancies 
and of other autoimmune disorders. We also provide a cross-reference to 
final 14.00E, the section of the introductory text that addresses 
immune deficiency disorders in detail.
    In final 14.00A4, Human immunodeficiency virus (HIV) infection, we 
provide a brief description of HIV infection. As in the NPRM, we 
include the first sentence from prior 14.00D1 in this section. However, 
in an editorial change from the prior rules and the NPRM, we have 
deleted the statement in the sentence that HIV infection is ``caused by 
a specific retrovirus.'' The change is not substantive, but only 
clarifies and updates our rules. It is now known that there are several 
forms of human immunodeficiency virus, therefore our statement that HIV 
infection is caused by ``a specific'' virus could be misleading. Also, 
since the ``V'' in the abbreviation ``HIV'' stands for ``virus,'' the 
sentence in the prior rules did not need to state that human 
immunodeficiency virus infection is caused by a virus. We have retained 
the rest of the sentence, which explains that HIV infection may be 
characterized by increased susceptibility to opportunistic infections, 
cancers, or other conditions. We also provide a cross-reference to 
final 14.00F, the section of the introductory text that addresses HIV 
infection in detail.

Final 14.00B--What information do we need to show that you have an 
immune system disorder?

    In final 14.00B, we incorporate the first sentence of the second 
paragraph of prior 14.00B to explain what information we need to show 
that you have an immune system disorder. We moved the second and third 
sentences of the second paragraph of prior 14.00B, which define our 
term ``appropriate medically acceptable imaging,'' to final 14.00C, a 
new section that provides definitions of terms in these listings. We 
are removing the last two sentences of the prior paragraph, which 
explained that we would not purchase tests that may involve significant 
risk. Since we already include this general policy in Sec. Sec.  
404.1519m and 416.919m of our regulations, it is not necessary to 
repeat it in this section. However, as we explain below, we are 
including guidance about the purchase of certain tests in other 
sections of these final rules.
    In the second sentence of final 14.00B, we provide that ``we will 
make every reasonable effort'' to obtain your medical history, medical 
findings, and the results of laboratory tests in documenting whether 
you have an immune system disorder. We included this requirement in 
prior 14.00D for HIV infection, but we did not include similar guidance 
in prior 14.00B for connective tissue disorders. We are adding this 
guidance under final 14.00B because it is appropriate for all immune 
system disorders.
    We also are removing the third and fourth paragraphs of prior 
14.00B. The third paragraph of prior 14.00B provided that we need a 
longitudinal clinical record of at least 3 months demonstrating active 
disease to assess the severity and duration of your impairment. This 
was not always the case, even under the prior rules. For example, 
individuals with HIV infection and cryptococcal meningitis (prior and 
final listing 14.08B4) or Kaposi's sarcoma (prior and final listing 
14.08E2), and individuals with ankylosing spondylitis with fixation 
(ankylosis) of the dorsolumbar spine at 45[deg] (prior listing 14.09B2, 
final listing 14.09C1) are disabled based on those findings alone. In 
these cases, we do not need 3 months of evidence or evidence showing 
active disease. Other cases may be decided with less than 3 months of 
evidence, while others may require more than 3 months of evidence. 
Therefore, we are removing this guidance because we must decide each 
case on an individual basis.

[[Page 14574]]

Final 14.00C--Definitions

    In final 14.00C, we define what we mean by important terms in these 
listings. As already noted, we include the definition of ``appropriate 
medically acceptable imaging'' from the second paragraph of prior 
14.00B. However, in an editorial change from the NPRM, we are revising 
the definition of ``appropriate'' imaging from ``one that is generally 
accepted and consistent with the prevailing state of medical knowledge 
and clinical practice'' to ``the proper one to support the evaluation 
and diagnosis of the impairment'' to be consistent with the language 
used in other body system listings, for example, the musculoskeletal 
body system (see 1.00C1) and hematological disorders body system (see 
7.00B). We are also including in this new section the definitions of 
the terms ``severe'' from the sixth paragraph of prior 14.00B, 
``inability to ambulate effectively'' and ``inability to perform fine 
and gross movements effectively'' from prior 14.00B6b, and ``resistant 
to treatment,'' ``recurrent,'' and ``disseminated'' from the second, 
third, and fourth paragraphs of prior 14.00D2. All of these terms apply 
to several, and sometimes all, of the final listings in this body 
system.
    In final 14.00C, we do not include the phrase ``must have lasted, 
or be expected to last, for at least 12 months'' from the definitions 
of ``inability to ambulate effectively'' and ``inability to perform 
fine and gross movements effectively'' that was in prior 14.00B6b 
because we believe it is unnecessary. Unless an impairment is expected 
to result in death, it must have lasted or must be expected to last for 
a continuous period of at least 12 months to meet the definition of 
disability. This change also makes the definitions of the terms 
consistent with the definitions of the same terms in 1.00B2b and 
1.00B2c in the musculoskeletal body system.
    We are also including, but simplifying, the definitions of the 
terms ``resistant to treatment,'' ``recurrent,'' and ``disseminated'' 
that were in prior 14.00D2, primarily to remove language that we 
believe was unnecessary. For example, we removed the explanation that 
the terms ``have the same general meaning as used by the medical 
community.'' These changes are editorial only, and the final 
definitions are not substantively different from the prior rules.
    In final 14.00C2, we are adding the definitions of several other 
important terms in these listings, including the term ``constitutional 
symptoms or signs.'' We are revising this definition slightly in 
response to a public comment on the NPRM to indicate that for purposes 
of these listings the constitutional symptoms or signs are severe 
fatigue, fever, malaise, and involuntary weight loss. In the proposed 
rules, we inadvertently referred to ``fatigue'' in our definition of 
constitutional symptoms or signs, rather than ``severe fatigue.'' We 
did, however, include a separate definition for ``severe fatigue'' 
because it is the criterion we use in all of the listings that include 
criteria for constitutional symptoms or signs. The change in the 
definition we are making in these final rules makes no substantive 
difference to the application of the listings, makes this definition 
consistent with the criteria of the listings, and more accurately 
reflects our intent.
    As in the NPRM, we are also providing a definition for the term 
``malaise.'' We are adding the definitions for severe fatigue and 
malaise in response to the many comments we received before we 
developed the proposed rules that indicated that the fatigue and 
malaise that people who have immune system disorders experience can be 
very limiting.
    In final 14.00C8, we reference current 1.00F for the definition of 
``major peripheral joints'' instead of restating the definition as we 
did in prior 14.00B6a.
    In final 14.00C12, we change ``describes'' to ``means.'' This is an 
editorial change from the NPRM for consistency with the other 
definitions in this section.

Final 14.00D--How do we document and evaluate the listed autoimmune 
disorders?

    We are changing the heading of proposed 14.00D in response to a 
public comment on the NPRM that we describe in the public comments 
section of this preamble. In final 14.00D, we are incorporating and 
expanding upon the information in prior 14.00B1 through 14.00B6, which 
described features commonly associated with each of the listed 
autoimmune system disorders. Throughout these sections, we refer to 
``autoimmune disorders'' instead of ``connective tissue disorders'' 
because the phrase ``autoimmune disorders'' is more medically accurate 
and more frequently used by medical professionals. We are also adding 
section 14.00D7 for Sjogren's syndrome because we are adding listing 
14.10 for that autoimmune disorder.
    In final 14.00D1, Systemic lupus erythematosus (14.02), we expand 
and clarify the information in prior 14.00B1. In final 14.00D1a, 
General, we explain that systemic lupus erythematosus (SLE) may involve 
any organ or body system and describe by body system some potential 
manifestations of SLE. We expand our explanation of how SLE is 
frequently characterized clinically. We are changing the reference to 
``fatigability'' used in prior 14.00B1 to ``severe fatigue'' to be 
consistent with how we describe the constitutional symptoms throughout 
the final immune system disorders listings. We are also adding 
``involuntary'' as a descriptor of weight loss to clarify that we mean 
weight loss due to SLE or its treatment, and to be consistent with our 
addition of this word throughout the introductory text and listings, as 
we have already explained.
    In final 14.00D1b, Documentation of SLE, we are updating our rules 
to explain that your medical evidence will generally, but not always, 
show that your SLE satisfies the criteria in the ``Criteria for the 
Classification of Systemic Lupus Erythematosus'' by the American 
College of Rheumatology, found in the most recent edition of the Primer 
on the Rheumatic Diseases published by the Arthritis Foundation. This 
is a more up-to-date reference than the 1982 reference in the prior 
rules.
    In final 14.00D2, Systemic vasculitis (14.03), we clarify the 
information in the prior rule. Final 14.00D2a, General, corresponds to 
the first three sentences of prior 14.00B2. In it, we explain what 
vasculitis is, and that it may be associated with other autoimmune 
disorders. We also give examples of several clinical patterns in which 
it may occur. We are removing the fourth sentence of prior 14.00B2, 
which described cutaneous vasculitis, because the impairment varies 
greatly in its manifestation, may not be associated with systemic 
involvement, and would not be expected to result in a listing-level 
impairment.
    Final 14.00D2b, Documentation of systemic vasculitis, corresponds 
to the last two sentences of prior 14.00B2. In it, we describe the 
documentation that is used to confirm the diagnosis of systemic 
vasculitis. In response to a comment described later in this preamble, 
we are expanding the guidance we provide in this section to explain 
that we will make ``every reasonable effort'' to obtain reports of 
angiography or tissue biopsy when they are part of your medical 
records. However, we will not purchase these invasive and costly 
procedures.
    Final 14.00D3, Systemic sclerosis (scleroderma) (14.04), 
corresponds to prior 14.00B3. We are revising the heading and expanding 
the information that was in the prior section. Final

[[Page 14575]]

14.00D3a, General, corresponds to the first three sentences of prior 
14.00B3. We are changing the term ``Raynaud's phenomena,'' which we 
used in the second and third sentences of prior 14.00B3, to ``Raynaud's 
phenomenon'' because the latter is the correct term. We make this same 
change in final listing 14.04C. In final 14.00D3b, Diffuse cutaneous 
systemic sclerosis, we continue to explain that, in addition to skin or 
blood vessels, major organ or systemic involvement may include the 
gastrointestinal tract, lungs, heart, kidneys, and muscle. This 
guidance corresponds to the fourth sentence in prior 14.00B3.
    Final 14.00D3c, Localized scleroderma (linear scleroderma or 
morphea), is new. We are adding this section and appropriate listings 
in final 14.04 for these disorders that originate in childhood because 
their disabling effects can persist into adulthood. Final 14.00D3c is 
essentially the same as final 114.00D3c, which we describe in detail 
later in this preamble. We are also making minor editorial changes from 
the language we proposed in the NPRM for clarity.
    Final 14.00D3d, Documentation of systemic sclerosis (scleroderma), 
is also new. In it, we explain what documenting systemic sclerosis 
(scleroderma) involves and that there may be an overlap with other 
autoimmune disorders.
    In final 14.00D4, Polymyositis and dermatomyositis (14.05), we 
clarify the information in prior 14.00B4. Final 14.00D4a, General, 
corresponds to the first three sentences of prior 14.00B4. It describes 
the characteristics of polymyositis and dermatomyositis. In the final 
rule, we have made minor editorial changes from the language we 
proposed in the NPRM.
    In final 14.00D4b, Documentation of polymyositis or 
dermatomyositis, we describe the findings that are generally used to 
document these impairments. The first sentence of the final rule 
corresponds to the last sentence of prior 14.00B4. We are making minor 
editorial revisions to the prior rules, including the removal of the 
reference to ``myositis,'' because there are multiple characteristic 
abnormalities on muscle biopsy that support the diagnosis of 
polymyositis or dermatomyositis. We also are adding a sentence to 
explain that people with dermatomyositis have characteristic skin 
findings. In response to a comment described later in this preamble, we 
are expanding the guidance we provide in this section to explain that 
we will make ``every reasonable effort'' to obtain reports of 
electromyography or muscle biopsy when they are part of your medical 
records. However, we will not purchase these procedures.
    In final 14.00D4c, Additional information about how we evaluate 
polymyositis and dermatomyositis under the listings, we explain how we 
evaluate commonly occurring limitations associated with these 
disorders. Final 14.00D4c(i) corresponds to the fourth and fifth 
sentences of prior 14.00B4. We are deleting the example of weakness of 
the anterior neck flexor muscles in the sixth sentence of prior 14.00B4 
because we are deleting the reference to the cervical muscles from 
listing 14.05 for reasons we explain later in this preamble. We are 
adding an example of rising independently from a squatting position 
because this is a common means for evaluating weakness in the pelvic 
girdle muscles.
    In final 14.00D4c(ii), we explain that we will evaluate 
malignancies (which may be associated with these disorders) under the 
malignant neoplastic diseases listings (13.00). (We do not provide this 
guidance in final 114.00D4c in the part B (childhood) section for 
polymyositis or dermatomyositis because malignancies are not commonly 
associated with these disorders in children.) We also explain that we 
evaluate the involvement of other organs or body systems under the 
affected body system.
    In final 14.00D5, Undifferentiated and mixed connective tissue 
disease (14.06), we reorganize and clarify the information from prior 
14.00B5. In the final rules, we are adding an explicit reference to 
mixed connective tissue disease (MCTD) to clarify what we meant in the 
prior rules when we referred to ``overlap'' syndromes. This is not a 
substantive change, but a clarification of our prior rules to update 
medical terminology. In final 14.00D5a, General, we describe what we 
mean by undifferentiated and mixed connective tissue disease. In final 
14.00D5b, Documentation of undifferentiated and mixed connective tissue 
disease, we explain when clinical features and serologic findings may 
be used to diagnose undifferentiated and mixed connective tissue 
disease. These provisions in final 14.00D5a and 14.00D5b are not 
substantively different from the provisions in the first three 
sentences of prior 14.00B5.
    We are removing the last sentence of prior 14.00B5. The sentence 
indicated that the correct designation of an ``overlap'' disorder is 
important for the assessment of prognosis. While the correct 
designation of an ``overlap'' disorder is useful in treatment settings, 
in our experience the requirement in our prior rules was not useful for 
adjudication.
    In final 14.00D6, Inflammatory arthritis (14.09), we expand, 
reorganize, and clarify the rules in prior 14.00B6. Throughout final 
14.00D6, we are simplifying the language of the NPRM, in which we used 
the rarely encountered word ``arthritides''; that is, the plural form 
of ``arthritis.'' Instead, we use the terms ``arthritis,'' and in final 
14.00D6a, ``the spectrum of inflammatory arthritis.''
    Final 14.00D6a, General, corresponds to the first and fourth 
sentences of prior 14.00B6. We continue to explain that inflammatory 
arthritis includes a vast array of disorders that differ in cause, 
course, and outcome, and that may result in difficulties with 
ambulation or fine and gross movements. We edited the fourth sentence 
of prior 14.00B6 to break it into three shorter sentences. However, we 
did not change the meaning of the provision. In addition to changing 
the term ``arthritides'' from the NPRM, we also made minor editorial 
changes in the final paragraph for clarity.
    Final 14.00D6b, Inflammatory arthritis involving the axial spine 
(spondyloarthropathy), and final 14.00D6c, Inflammatory arthritis 
involving the peripheral joints, correspond to the second and third 
sentences of prior 14.00B6. In these sections, we list some disorders 
that may be associated with inflammatory arthritis involving the axial 
spine (final 14.00D6b) and inflammatory arthritis affecting the 
peripheral joints (final 14.00D6c). We are including inflammatory bowel 
disease (IBD) in the lists of examples of specific disorders in these 
sections because arthritis is the most common extra-intestinal 
complication of IBD. In final 14.00D6b, we are not including the 
examples of ``other reactive arthropathies'' and ``undifferentiated 
spondylitis,'' which were in the second sentence of prior 14.00D6, 
because they are non-specific and we do not intend to provide a 
complete list, only some examples. Finally, we are updating some of the 
terminology in this section. For example, we refer to ``psoriatic 
arthritis'' instead of ``psoriatic arthropathy.''
    Final 14.00D6d, Documentation of inflammatory arthritis, is new. In 
it, we explain that generally, but not always, the diagnosis of 
inflammatory arthritis is based on the clinical features and serologic 
findings described in the most recent edition of the Primer on the 
Rheumatic Diseases.
    Final 14.00D6e, How we evaluate inflammatory arthritis under the 
listings, corresponds to the information

[[Page 14576]]

in the last two sentences of prior 14.00B6, prior 14.00B6c, and prior 
14.00B6d. We are reorganizing the text to reflect the reorganization of 
listing 14.09, which we explain later in this preamble, and to clarify 
it. We are also making changes to 14.00D6e in response to a public 
comment on the NPRM, as explained below and in the public comments 
section of this preamble.
     Final 14.00D6e(i) explains that final listings 14.09A and 
14.09C1 (prior listings 14.09A and 14.09B) are met by showing an 
impairment that results in an ``extreme'' limitation. This is how we 
describe ``inability to ambulate effectively'' in 1.00B2b in our 
musculoskeletal listings and, therefore, it is only a clarification of 
the prior rule. In the final rule, we retain the provision from prior 
14.00B6c that the inability to ambulate effectively is implicit in 
final listing 14.09C1 (prior listing 14.09B), the listing for ankylosis 
of the spine with fixation at a 45[deg] angle, even though individuals 
who have the degree of ankylosis described in the listing ordinarily do 
not require the use of bilateral upper limb assistance.
    A public commenter on the NPRM pointed out that proposed (and 
prior) listing 14.09 did not account for individuals who are unable to 
ambulate effectively because of involvement of a major peripheral joint 
in one lower extremity, requiring our adjudicators to refer to listings 
1.02 and 1.03 in those cases. In response to this comment, we decided 
to simplify our rules so that there is no longer a need to cross-refer 
to the listings in the musculoskeletal system. We revised listing 14.09 
(and listing 114.09) so that all individuals with inflammatory 
arthritis who are unable to ambulate effectively or to use their upper 
extremities effectively can qualify under the inflammatory arthritis 
listing. As a consequence, we revised this section to reflect the 
revised listing criteria. We also removed proposed 14.00D6e(iv) and 
14.00D6e(v) as explained below. (For clarity, we are also revising a 
sentence in 1.00B1 and 101.00B1 in the musculoskeletal system listings. 
We describe this and the public comment that led to these changes in 
the public comments section of this preamble.)
     Final 14.00D6e(ii) explains final listings 14.09B (prior 
listing 14.09D), 14.09C2 (prior listing 14.09E), and 14.09D. We revised 
the language in the NPRM to more clearly explain that listing-level 
severity can result from various combinations of complications from 
inflammatory arthritis. This is not a substantive change, only a 
clarification. In this section, we also incorporate the provision in 
the first sentence of prior 14.00B6d that extra-articular impairments 
may meet listings in other body systems.
     Final 14.00D6e(iii) corresponds to the third and fourth 
sentences of prior 14.00B6d. It explains that extra-articular features 
of inflammatory arthritis may involve any body system and lists 
examples of commonly occurring extra-articular impairments by body 
system. We are reorganizing and expanding the list of examples of such 
impairments from the prior rules and clarifying the body systems to 
which they belong. We are also making a minor editorial change to the 
sentence we proposed. In the NPRM, we introduced the list of examples 
with the statement ``Commonly occurring extra-articular impairments 
include * * *.'' However, the list that followed was actually a list of 
body systems, each of which contained parenthetical examples of 
specific impairments. In the final rules, we are providing a more 
accurate introduction to the list of examples of body systems and their 
parenthetical examples.
     As indicated above, we removed proposed 14.00D6e(iv) and 
14.00D6e(v) in response to a public comment. These sections 
corresponded to the last sentence of prior 14.00B6, which explained 
that we used listing 1.02 or 1.03 in the musculoskeletal system when 
the dominant feature of the impairment was persistent deformity without 
ongoing inflammation or when there had been surgical reconstruction.
     Final 14.00D6e(iv) (proposed 14.00D6e(vi)) clarifies that 
we evaluate your impairment under any appropriate listing when you have 
both inflammation and chronic deformities.
    We are not including the provisions of prior 14.00B6e in these 
final rules. Prior 14.00B6e provided that the fact that an individual 
is dependent on steroids, or any other drug, for the control of 
inflammatory arthritis is insufficient in itself to establish 
disability. We added it to part A of our listings in 2002 for 
consistency with 114.00E6, a provision we added to part B of the 
listings at the same time (66 FR at 58020 (2001)). We are removing that 
provision for reasons we explain below in our summary of the final 
rules in part B. Therefore, we are removing this provision in part A 
for consistency with that change. However, in final 14.00G3, we 
continue to state that we will consider the adverse side effects of 
treatment, including the adverse effects of corticosteroids, to ensure 
that our adjudicators consider the side effects an individual might 
experience from steroids and any other treatment.
    Final 14.00D7, Sj[ouml]gren's syndrome (14.10), is new. As already 
noted, we are adding a listing for Sj[ouml]gren's syndrome. In 
connection with that final listing, final 14.00D7a, General, explains 
the features of the disorder, including its resulting symptoms and 
possible complications. We also list organ systems that may be involved 
and note that Sj[ouml]gren's syndrome may be associated with other 
autoimmune disorders. In final 14.00D7b, Documentation of 
Sj[ouml]gren's syndrome, we also explain that if you have 
Sj[ouml]gren's syndrome, your medical evidence will generally, but not 
always, show that your disease satisfies the criteria in the current 
``Criteria for the Classification of Sj[ouml]gren's Syndrome'' found in 
the most recent edition of the Primer on the Rheumatic Diseases.

Final 14.00E--How do we document and evaluate immune deficiency 
disorders, excluding HIV infection?

    We changed the heading of proposed 14.00E in response to a public 
comment on the NPRM that we describe in the public comments section of 
this preamble. In final 14.00E, we add a section describing how immune 
deficiency disorders (excluding HIV infection) are classified, 
documented, and evaluated. This section has four subsections.
     In final 14.00E1, General, we explain that immune 
deficiency disorders are classified as either ``primary'' or 
``acquired.'' Primary disorders are mainly seen in children but, due to 
recent advances in treatment, many affected children survive into 
adulthood.
     In final 14.00E2, Documentation of immune deficiency 
disorders, we explain that documentation of these disorders may be 
based on laboratory evidence or by other generally acceptable methods 
consistent with the prevailing state of medical knowledge and clinical 
practice.
     In final 14.00E3, Immune deficiency disorders treated by 
stem cell transplantation, we explain how we evaluate immune deficiency 
disorders that are treated in this way. In final 14.00E3a, Evaluation 
in the first 12 months, we explain that if you undergo stem cell 
transplantation, we will consider you disabled until at least 12 months 
from the date of the transplant. This is the same provision that we use 
for most malignancies treated by bone marrow or stem cell transplants 
in the neoplastic listings. In 13.00L3b of the malignant neoplastic 
diseases body system, we also include a special provision for 
autologous bone marrow transplants--transplants using your own

[[Page 14577]]

stem cells. We do not include such an alternative provision in these 
final rules because people with immune deficiency disorders receive 
allogeneic transplants--that is, stem cells taken from other people. 
Also, unlike in the rules in the malignant neoplastic diseases body 
system, we use the phrase ``stem cell transplantation'' instead of 
``bone marrow or stem cell transplantation'' in this final section and 
in final listing 14.07B because ``stem cell transplantation'' is a 
broader term that encompasses different sites for obtaining 
hematopoetic (blood-forming) stem cells, including bone marrow, 
peripheral blood, and umbilical cord blood. In final 14.00E3b, 
Evaluation after the 12-month period has elapsed, we explain that after 
this period has elapsed, we consider any demonstrable residuals of your 
immune deficiency disorder including any residual impairment(s) 
resulting from your treatment. The provision is based on 13.00L4 in our 
malignant neoplastic diseases listings.
     In final 14.00E4, Medication-induced immune suppression, 
we explain that medication can result in immune suppression that will 
usually resolve once the medication is ceased. However, if you take 
prescribed medications for long-term immune suppression, such as after 
an organ transplant, we will look at the frequency and severity of any 
infections you get, residuals from the organ transplant itself, and 
whether there has been any significant deterioration of other organ 
systems.

Final 14.00F--How do we document and evaluate human immunodeficiency 
virus (HIV) infection?

    We changed the heading of proposed 14.00F in response to a public 
comment on the NPRM that we describe in the public comments section of 
this preamble. In final 14.00F, we incorporate, update, and expand 
information on HIV infection that was contained in prior 14.00D3 
through 14.00D7. We also make nonsubstantive editorial changes.
    As already noted, we moved the first sentence of prior 14.00D1 to 
final 14.00A4. Therefore, we begin final 14.00F with the second 
sentence of prior 14.00D1. It is a reminder that an individual's HIV 
infection need not meet the Centers for Disease Control and Prevention 
(CDC) definition of acquired immune deficiency syndrome (AIDS) to meet 
or medically equal the criteria of listing 14.08. We made minor 
editorial changes to the sentence, but did not change its meaning.
    We do not require an individual's HIV infection to meet the CDC 
definition of AIDS because in evaluating disability claims, our concern 
is to determine whether an individual's impairment(s) is severe enough 
to prevent him or her from engaging in any substantial gainful 
activity. The CDC's definition is designed to enhance its capability 
for activities such as disease reporting and surveillance, 
epidemiologic studies, prevention and control activities, and public 
health policy and planning. This definition is not intended to 
determine whether any statutory or regulatory requirements for 
disability are met.
    We moved the provisions of prior 14.00D2 to other sections in the 
final rules. In the first four paragraphs of prior 14.00D2, we defined 
the terms ``resistant to treatment,'' ``recurrent,'' and 
``disseminated,'' and we now define those terms in final 14.00C. In the 
fifth paragraph of prior 14.00D2, we defined ``significant involuntary 
weight loss'' for purposes of prior listing 14.08I (final listing 
14.08H). In the final rules, we include this definition in 14.00F5.
    Like prior 14.00D3, final 14.00F1 is in two major sections: A 
section explaining how we document the diagnosis of HIV infection 
definitively (14.00F1a) and a section explaining how we document the 
diagnosis of HIV infection when we do not have definitive evidence 
(14.00F1b). In final 14.00F1, Documentation of HIV infection, we 
incorporate and update the information in prior 14.00D3 to explain the 
laboratory tests or other evidence we accept as documentation of HIV 
infection. In response to a public comment on the NPRM, we have also 
added a statement, similar to the statements we added in final 14.00D2b 
and 14.00D4b, explaining that we will not purchase laboratory testing 
to establish whether you have HIV infection.
    Final 14.00F1a, Definitive documentation of HIV infection, 
corresponds to prior 14.00D3a. We updated and expanded this section to 
include newer laboratory diagnostic techniques that did not exist or 
were not widely used when we published the prior rules in 1993.
     Final 14.00F1a(i), for HIV antibody tests, corresponds to 
prior 14.00D3a(i). We made only nonsubstantive editorial changes.
     Final 14.00F1a(ii) is new from our prior rules. It adds 
positive ``viral load'' tests for HIV infection, such as quantitative 
plasma HIV RNA, quantitative plasma HIV branched DNA, and reverse 
transcriptase-polymerase chain reaction (RT-PCR), that were not widely 
available when we published the prior rules.
     Final 14.00F1a(iii) is for HIV DNA detection by polymerase 
chain reaction (PCR). We included it as an example of an ``other test'' 
in prior 14.00D3a(iii) because it was not widely available when we 
published the prior rules.
     Final 14.00F1a(iv), for HIV antigen, corresponds to prior 
14.00D3a(ii).
     Final 14.00F1a(v) is new from our prior rules. It adds a 
positive viral culture for HIV from peripheral blood mononuclear cells 
(PBMC) as another test that definitively documents HIV infection. Even 
though it is not commonly used, we will accept it as definitive 
evidence if it is in your medical records.
     Final 14.00F1a(vi), for other tests that are highly 
specific for detection of HIV, corresponds to the first paragraph in 
prior 14.00D3a(iii).
    Final 14.00F1b, Other acceptable documentation of HIV infection, 
corresponds to prior 14.00D3b. It explains what documentation of HIV 
infection we will accept instead of definitive laboratory testing. The 
final rule is essentially the same as the prior rule except for 
nonsubstantive editorial changes. However, in response to a public 
comment on the NPRM, we removed the word ``carinii'' and refer now only 
to ``Pneumocystis pneumonia'' (PCP) in this section and others in these 
final rules. We explain the reason for this change in the public 
comments section of this preamble.
    In final 14.00F2, CD4 tests, we combine the provisions in the 
second undesignated paragraph after prior 14.00D3a(iii) and the second 
paragraph in prior 14.00D4a. We specify that, even though a reduced CD4 
count or percent alone does not establish a definitive diagnosis of HIV 
infection, a count below 200/mm\3\ (or below 14 percent of the total 
lymphocyte count) along with clinical findings does offer supportive 
evidence of the existence of HIV infection without a definitive 
diagnosis. This is because a CD4 count below 200 is an indicator of an 
increased susceptibility to developing opportunistic infections.
    In the final rules, we slightly revised the language we proposed to 
correct minor inconsistencies in the NPRM. In the fourth sentence of 
proposed 14.00F2, we referred to a CD4 count ``below 200.'' However, in 
the third sentence, we referred to a CD4 count that is ``200 mm\3\ or 
less,'' which is not precisely the same thing. In these final rules, we 
are correcting the third sentence to also say ``below 200'' for 
consistency. Likewise, we revised the parenthetical reference to 
``below 14

[[Page 14578]]

percent'' and clarified that the reference is to the percentage of CD4 
cells to the total lymphocyte count. We made the same changes 
throughout these final rules for consistency with these corrections. We 
also made nonsubstantive editorial changes in this paragraph.
    In final 14.00F3, Documentation of the manifestations of HIV 
infection, we incorporate the information in prior 14.00D4 with 
nonsubstantive editorial changes. Like final 14.00F1 and prior 14.00D4, 
final 14.00F3 is divided into two main parts:
     Final 14.00F3a, Definitive documentation of the 
manifestations of HIV infection, incorporates the first paragraph in 
prior 14.00D4a and explains how we document manifestations of HIV 
infection definitively.
     Final 14.00F3b, Other acceptable documentation of the 
manifestations of HIV infection, incorporates information that was in 
the first paragraph of prior 14.00D4b and explains how we document 
manifestations of HIV infection when we do not have definitive 
evidence.
    We are revising the language of proposed 14.00F3b to clarify our 
original intent. In the prior rule, we indicated that ``if no 
definitive laboratory evidence is available, manifestations of HIV 
infection may be documented by medical history, clinical and laboratory 
findings, and diagnosis(es) indicated in the medical evidence.'' The 
sentence may have implied that we needed to have all of the things 
listed (medical history and clinical findings and laboratory findings 
and diagnosis(es)) to determine that you have a manifestation of HIV 
infection when we do not have definitive laboratory findings. That was 
not our intent, so we are clarifying in the final rule that we may need 
only some of this information to make a finding that you have a 
manifestation of HIV infection, depending on the prevailing state of 
medical knowledge and clinical practice. We are also clarifying what we 
mean by ``laboratory findings'' in this context; that is, laboratory 
findings that do not in themselves definitively establish the existence 
of an HIV-related manifestation. In response to a public comment on the 
NPRM, we are also clarifying in final 14.00F3b that the manifestations 
that are listed are only examples of manifestations that can be 
diagnosed without definitive evidence. We will accept a presumptive 
diagnosis of any manifestation of HIV infection so long as the method 
used to make the diagnosis is consistent with the prevailing state of 
medical knowledge and clinical practice.
    In 14.00D4 of the prior rules we provided specific guidance for 
documenting one particular manifestation of HIV infection without 
definitive evidence: Cytomegalovirus (CMV) disease. In final 14.00F3b, 
we expand the section to include three additional manifestations, 
including a manifestation we added in response to a public comment on 
the NPRM. The revised guidance is as follows:
     In final 14.00F3b(i), we explain that PCP is frequently 
diagnosed presumptively without definitive evidence and provide 
examples of evidence that is supportive of a presumptive diagnosis of 
PCP. Because we removed the word ``carinii'' in a change we made in 
final 14.00F1b, we no longer need the parenthetical note we proposed to 
include in 14.00F3b(i); therefore, we have not included it in these 
final rules. In response to a public comment on the NPRM, we also added 
``no evidence of bacterial pneumonia'' to the list of evidence that is 
supportive of a presumptive diagnosis of PCP. For consistency with a 
change we made in final 14.00F3b(ii) in response to a public comment on 
the NPRM, we also indicate that supportive evidence of a presumptive 
diagnosis of PCP ``may'' include the items we list. This is not a 
change in the meaning of the proposed rule, only a clarification.
     In final 14.00F3b(ii), we incorporate and expand the 
information now in the second paragraph of prior 14.00D4b, regarding 
the documentation of CMV disease. However, in an editorial change from 
the NPRM, we revised the second and fourth sentences and removed the 
third sentence in proposed 14.00F3b(ii). In the NPRM, we stated that a 
serology test ``identifies a history of infection with CMV, but it does 
not confirm an active disease process.'' We revised this to state that 
a serology test ``does not establish a definitive diagnosis of CMV 
disease, but it does offer supportive evidence of a presumptive 
diagnosis of CMV disease.'' Due to this revision, we removed a positive 
CMV serology test from the list of examples of clinical findings that 
are supportive of a presumptive diagnosis of CMV that were in the 
fourth sentence of the proposed section, and revised the sentence to 
indicate that the examples provided are other clinical findings that 
support a presumptive diagnosis of CMV. We removed the third sentence 
because it was unnecessary. These changes are not substantive, only a 
clarification of the proposed rules. As in the NPRM, we do not include 
``documentation of CMV disease requires confirmation by biopsy'' as in 
the last sentence of the second paragraph of prior 14.00D4b because we 
are providing information on documentation other than definitive 
laboratory findings. Also, instead of stating that we can use generally 
acceptable methods to confirm the diagnosis of CMV, we provide examples 
of evidence, such as fever and a positive CMV serology test, that is 
supportive of a presumptive diagnosis of CMV disease. In response to a 
public comment on the NPRM, we are clarifying that an individual need 
not have all of the findings we list by indicating that supporting 
evidence ``may'' include these findings.
     In final 14.00F3b(iii), we explain how toxoplasmosis of 
the brain is presumptively diagnosed since the definitive method of 
diagnosing toxoplasmosis of the brain by biopsy is not commonly 
performed.
     In final 14.00F3b(iv) we provide guidance about how 
candidiasis of the esophagus may be presumptively diagnosed. We explain 
our reasons for making this addition and the other changes summarized 
above in the public comments section of this preamble.
    We are also making a minor change from the NPRM in the opening 
paragraph of 14.00F3. The last sentence explained that we will make 
every reasonable effort to obtain reports of the results of laboratory 
testing you have had for a manifestation of HIV infection. We are not 
including that sentence in final 14.00F3 because it is repetitive of 
other provisions in these final rules and in our other regulations. 
See, for example, final 14.00B and current Sec. Sec.  404.1512 and 
416.912. Therefore, this revision is only editorial, simplifying the 
proposed rule without changing any requirements.
    In final 14.00F4, HIV infection manifestations specific to women, 
we incorporate the information in prior 14.00D5. In final 14.00F4a, 
General, we incorporate the first paragraph of prior 14.00D5, while in 
final 14.00F4b, Additional considerations for evaluating HIV infection 
in women, we incorporate the second paragraph of prior 14.00D5. Except 
for adding paragraph designations and headings and minor editorial 
changes (including changes that are reflected in the paragraph 
designations of the listings explained below), the final provisions are 
the same as in the prior rules.
    In final 14.00F5, Involuntary weight loss, we incorporate the last 
paragraph of prior 14.00D2 with nonsubstantive editorial changes, 
including a change that reflects the redesignation of prior

[[Page 14579]]

listing 14.08I as final listing 14.08H. In a change from the NPRM, we 
are not including the first sentence we had proposed, which was also in 
the prior rules. The sentence said, `` `[S]ignificant involuntary 
weight loss' does not correspond to a specific minimum amount or 
percentage of weight loss.'' The sentence could have been confusing 
because the very next sentence (what is now the first sentence in the 
final rule) explains that a 10 percent weight loss is always 
``significant''; therefore, in some cases ``significant weight loss'' 
does correspond to a specific percentage. It was also unnecessary 
because the next sentence (the second sentence in the final rule) 
explains that a weight loss of less than 10 percent may or may not be 
``significant,'' which has essentially the same meaning as the sentence 
we removed.

Final 14.00G--How do we consider the effects of treatment in evaluating 
your autoimmune disorder, immune deficiency disorder, or HIV infection?

    In final 14.00G, we explain how we consider the effects of 
treatment for all three categories of immune system disorders; that is, 
autoimmune disorders, immune deficiency disorders, and HIV infection. 
The new section addresses in one place issues of treatment that are 
common to all three types of immune system disorders as well as issues 
of treatment that are unique to each type of disorder, including 
treatment that is specifically for HIV infection. We did not remove any 
guidance about treatment for HIV infection that is still relevant, but 
instead we moved it to this new section. In fact, we expanded and 
updated our rules to reflect what has been learned in applying 
different treatments for HIV infection since we published the prior 
rules. The provisions for addressing both the positive effects and 
negative side effects of treatment in individuals who have autoimmune 
disorders and immune deficiency disorders, other than HIV infection, 
are new in these final listings and, we believe, provide useful 
adjudicative guidance that was lacking in the prior rules.
    Final section 14.00G has six subsections. The first two (final 
14.00G1 and 14.00G2) and the last one (final 14.00G6) are applicable to 
all immune system disorders. Final 14.00G3-14.00G5 provide guidance 
specific to each of the three main types of immune system disorders: 
Autoimmune disorders (final 14.00G3), immune deficiency disorders, 
excluding HIV infection (final 14.00G4), and HIV infection (final 
14.00G5).
    In final 14.00G1, General, we incorporate the first and fifth 
sentences of prior 14.00D7. We believe that this guidance has general 
applicability to all immune system disorders, not just HIV infection. 
We first explain that we consider the effectiveness of your treatment 
on your signs, symptoms, and laboratory findings, and the negative side 
effects of your treatment on your functioning. We also explain that we 
will make every reasonable effort to obtain a specific description of 
the treatment you receive. Then, we list eight factors we consider when 
we evaluate your treatment. They are mostly based on factors we 
mentioned in the prior rule, but we expanded the list, and in some 
cases clarified the factors that were in the prior rules. For example, 
instead of referring only to the ``dosage [and] frequency of 
administration'' of your treatment, we refer to ``the intrusiveness and 
complexity of your treatment (for example, dosing schedule, need for 
injections).'' In final 14.00G1e, we also introduce the term 
``variability of your response to treatment,'' a concept we addressed 
for HIV infection in prior 14.00D7 but that we believe is of particular 
importance in considering the effects of treatment in all individuals 
with immune system disorders. We explain this concept in more detail in 
final 14.00G2.
    Final 14.00G1f is new. It describes the interactive and cumulative 
effects of treatments for immune system disorders and other disorders 
that persons with immune system disorders may also have. We explain 
that the effects of these treatments taken together may be greater than 
they would be if we considered them separately, and we provide an 
example of treatment for HIV infection together with treatment for 
hepatitis C. Final 14.00G1g is also new. It explains that we will also 
consider the duration of your treatment. Final 14.00G1h is a catchall 
for other relevant factors we have not listed in 14.00G1a-14.00G1g.
    In final 14.00G2, Variability of your response to treatment, we 
explain what we mean by this factor in terms of both HIV infection and 
other immune system disorders. The final rule is based on the language 
of the second paragraph in prior 14.00D7 and the second sentence of the 
third paragraph of that section. However, we are expanding that 
guidance and applying it to all other immune system disorders in 
addition to HIV infection. For example, we explain in a general way 
applicable to all immune system disorders that some individuals may 
show an initial positive response to drug treatment (or a combination 
of drugs), but the initial positive response may be followed by a 
decrease in the effectiveness of the medication.
    We provide more specific information about treatment of autoimmune 
disorders in final 14.00G3, How we evaluate the effects of treatment 
for autoimmune disorders on your ability to function. This final rule 
repeats the rule in the fifth paragraph of prior 14.00B that we 
consider the adverse effects that may result in loss of function when 
we evaluate the effects of your treatment for your autoimmune 
disorder(s). We expanded this guidance to include more examples of 
potential chronic adverse effects of steroid treatment and to explain 
that the side effects of some medications may be acute or long-term. We 
add a provision that recognizes that the medications used in the 
treatment of autoimmune disorders may have effects on mental function, 
including cognition (memory), concentration, and mood.
    Final 14.00G4, How we evaluate the effects of treatment for immune 
deficiency disorders, excluding HIV infection, on your ability to 
function, is new. As in final 14.00G3, we repeat the principle that we 
will consider the side effects of your treatment when we evaluate your 
ability to function. We cite intravenous immunoglobulin and gamma 
interferon therapy as examples of treatment you may be receiving. We 
also provide examples of side effects of treatment for immune 
deficiency disorders, including physical symptoms (such as severe 
fatigue and headaches), clinical signs (such as high blood pressure and 
joint swelling), or limitations in mental function, including 
cognition, concentration, and mood.
    Final 14.00G5, How we evaluate the effects of treatment for HIV 
infection on your ability to function, is in two parts. In final 
14.00G5a, General, as in final 14.00G3 and 14.00G4, we repeat the 
principle from prior 14.00D7 that we consider the side effects of 
antiretroviral treatment and treatment for the manifestation of HIV 
infection on your ability to function. We expand the guidance to 
provide examples of the physical and mental side effects of 
antiretroviral drugs. We also note that the symptoms of HIV infection 
and the side effects of medications may be indistinguishable, and that 
we will consider your functional limitations whether they are a result 
of your symptoms or signs of HIV infection or the side effects of your 
treatment.
    We made two changes in final 14.00G5a in response to a public 
comment on the NPRM. We added a parenthetical reference to ``fat

[[Page 14580]]

redistribution, such as `buffalo hump'.'' ``Fat redistribution'' is 
another name for lipodystrophy, which we had included in the proposed 
rule, and ``buffalo hump'' is a kind of lipodystrophy. We also expanded 
the last sentence of the paragraph to explain that we consider 
functional limitations from signs of HIV infection as well as from 
symptoms. We explain our reasons for these changes in the public 
comments section of this preamble.
    In final 14.00G5b, Structured treatment interruptions, we provide 
new guidance specifically about structured treatment interruptions 
(STIs, also called drug holidays) in individuals with HIV infection. 
The guidance explains that STIs are part of a prescribed treatment 
plan; therefore, they do not show that an individual is failing to 
follow treatment or in themselves establish that an individual's 
impairment is not as severe as alleged.
    In final 14.00G6, When there is no record of ongoing treatment, we 
explain how we evaluate the medical severity and duration of your 
immune system disorder when you have not received ongoing treatment or 
have not had an ongoing relationship with any treatment source despite 
the existence of a severe impairment(s). The provision is based on a 
standard provision we include in most other body system listings; for 
example, 1.00H3 in the musculoskeletal system, the third paragraph of 
3.00A in the respiratory system, and the third paragraph of 4.00B3 in 
the cardiovascular system. We also explain that if you have just begun 
treatment and we cannot decide whether you are disabled based on the 
evidence we have, we may need to wait to determine the effect of your 
treatment. We explain that there is no set period because how long we 
may need to wait will depend on the facts of your individual case. This 
is consistent with the guidance we provided in the last two sentences 
of the third paragraph in prior 14.00D7, which explained that decisions 
about the impact of treatment should be based on a sufficient period of 
treatment to permit proper consideration of the temporary or long-term 
effects of the treatment.

Final 14.00H--How do we consider your symptoms, including your pain, 
severe fatigue, and malaise?

    Final 14.00H is new. In it, we explain that we will evaluate the 
impact your symptoms have on your ability to function when the evidence 
of your immune system disorder(s) shows that you have a medically 
determinable impairment that could reasonably be expected to produce 
your symptoms.
    We added a sentence in the final rule in response to a public 
comment we describe later in this preamble. The sentence explains that 
we will not draw any inferences about your symptoms and their 
functional effects from the fact that you do not receive treatment or 
you are not following treatment without considering all of the relevant 
evidence in your case record, including any explanations you provide 
that may explain why you are not receiving or following treatment. As 
we explain in more detail later, the sentence is based on a provision 
in Social Security Ruling (SSR) 96-7p. We also clarified the heading in 
the final rule by listing the two constitutional symptoms, severe 
fatigue and malaise, instead of referring to ``constitutional 
symptoms.''

Final 14.00I--How do we use the functional criteria in these listings?

    We indicated in the ANPRM that we would not summarize or respond to 
the public comments (68 FR 24897). However, there was one theme that 
was common to many of the letters and e-mails and that was raised 
repeatedly at our two outreach meetings by the medical specialists, 
advocates for persons who have immune system disorders, and individuals 
with immune system disorders: The functional impact of immune system 
disorders, and the inadequacy of the immune system rules to address 
that impact, especially for immune system disorders other than HIV 
infection. This issue was raised so often, and as a matter of such 
great public interest, that we believe that it will be helpful to 
summarize briefly what commenters said to help explain why we are 
adding new rules for evaluating functioning in these listings.
    Many commenters said that we should recognize how immune system 
disorders can affect an individual's functioning. Many individuals 
described physical symptoms, such as pain, fatigue, and malaise, as 
well as mental symptoms, including loss of memory, loss of 
concentration, and depression. Commenters stressed that these symptoms 
could be very severe. A number of persons indicated that the fatigue 
associated with these disorders was not merely a feeling of tiredness 
but a more profound and debilitating experience. Many individuals also 
noted that the impairments could be both episodic and variable in 
intensity, with some individuals experiencing ``good'' or relatively 
good days interspersed with days in which they were unable to function. 
They pointed out that there was a need for the rules to recognize the 
longitudinal effect of these episodic limitations on the ability to 
work. Other persons pointed out that there is often comorbidity of 
immune system disorders, that is, many persons have features of more 
than one immune system disorder. In those cases, the combination of 
symptoms and limitations have a multiplication effect in the 
individual's overall condition that is worse than simply adding the 
individual effects of the symptoms and limitations to each other. These 
commenters said that under the prior listings there is no adequate way 
to assess these multiplied effects. Many commenters also pointed out 
the effect that stress can have on the medical condition and 
symptomatology of individuals who have immune system disorders. Other 
individuals described the debilitating effects of treatment, not only 
the side effects, but sometimes the need to follow a very rigorous and 
time-consuming schedule of treatment that in itself can be limiting.
    A number of the commenters pointed with approval to the provisions 
of prior listing 14.08N and the text in prior 14.00D8 that explains 
that listing. These individuals thought that the provisions should not 
be confined to persons who have HIV infection but should be extended to 
individuals with other kinds of immune system disorders who may be 
continuously limited by their symptoms and other manifestations, 
frequently become ill, have periodic manifestations, or have the kinds 
of serious limitations described in those rules. They urged us to 
consider extending such criteria to all listed immune system disorders 
to ensure that we do not overlook individuals who do not necessarily 
have the objective evidence needed to meet the other criteria in the 
listings but who may still be disabled.
    As we have noted, in these final rules we are significantly 
expanding our guidance about specific immune system disorders and the 
effects of treatment. We also agree with those commenters on the ANPRM 
and at the public outreach meetings who suggested that we include the 
same kind of criteria for evaluating the overall functional impact of 
other immune system disorders as we provided in prior listing 14.08N 
for persons who have HIV infection. Therefore, we are adding criteria 
similar to those in prior listing 14.08N (final listing 14.08K) for 
each of the listed impairments in this body system. The final listings 
for evaluating functioning for other immune system disorders are 
14.02B, 14.03B, 14.04D, 14.05E, 14.06B, 14.07C, 14.09D, and 14.10B. We 
are also redesignating prior listing 14.08N as

[[Page 14581]]

final 14.08K for reasons we explain below.
    Final 14.00I is the section of the introductory text that explains 
the listings that include functional criteria. It corresponds to prior 
14.00D8, but we revised it so that it applies to all of the new final 
listings that include functional criteria, not just the listing for HIV 
infection (prior listing 14.08N).
    Like prior 14.00D8, final 14.00I includes eight paragraphs. Except 
as described below, we revised each paragraph so that it applies not 
only to HIV infection but to the other immune system disorders as well. 
For example, in the first paragraph of prior 14.00D8 we explained that 
prior listing 14.08N (final listing 14.08K) established standards for 
evaluating manifestations of HIV infection that do not meet the 
criteria of any of the preceding listings within 14.08; that is, prior 
listings 14.08A-14.08M. We also explained that we used prior listing 
14.08N both for manifestations that were listed in the preceding 
listings within 14.08 and for manifestations that were not listed at 
all. We have modified this language so that it applies to all of the 
immune system disorders within this body system. We also made minor 
editorial changes throughout the paragraphs.
    The following are other changes we are making in this section.
    In final 14.00I2, we are removing the first sentence in the second 
paragraph of prior 14.00D8. That sentence explained that, for 
individuals with HIV infection, we assessed listing-level severity 
under prior listing 14.08N based on the functional limitations imposed 
by the impairment. We believe that this point is already made in final 
14.00I1 and that it is unnecessary to repeat it in final 14.00I2. We 
are revising the second sentence, which said that we must consider the 
full impact of ``signs, symptoms, and laboratory findings'' on the 
individual's ability to function. We believe that this guidance may not 
have clearly explained what we intended. Therefore, we are revising it 
to explain that when we use one of the listings cited in final 14.00I1, 
we will consider all relevant information in your case record to 
determine the full impact of your immune system disorder(s) on your 
ability to function on a sustained basis.
    In final 14.00I3-14.00I8, which correspond to the last six 
paragraphs in prior 14.00D, we are updating our rules to make their 
language more consistent with our other rules that define the term 
``marked'' and the areas of functioning. However, these changes are not 
intended to be substantively different from the prior rules. We are 
also including references to both pain and severe fatigue throughout 
final 14.00I6-14.00I8 as symptoms that may cause limitations. The prior 
rules were not consistent in this regard.
    We added guidance in final 14.00I3 in response to public comments 
on the NPRM. The guidance clarifies that your impairment will satisfy 
the criterion for ``repeated'' manifestations regardless of whether you 
have the same kind of manifestation repeatedly, all different 
manifestations, or a combination of some manifestations that are the 
same and some different; for example, two of the same kind of 
manifestation and one different one. You must only have the required 
number of manifestations with the frequency and duration required in 
this section. This is not a change in meaning from the proposed rules, 
but a clarification of our intent. In response to another comment, we 
also clarify that the manifestations must occur within the period 
covered by your claim.

Final 14.00J--How do we evaluate your immune system disorder when it 
does not meet one of these listings?

    Final 14.00J1 and 14.00J3 replace the guidance we provided in the 
first and third paragraphs of prior 14.00D6. As in other provisions 
throughout the introductory text, we are revising the language to make 
it apply generally to all immune system disorders, not just HIV 
infection. Also, we are removing guidance that is already covered in 
other sections in the introductory text, such as the guidance that 
individuals may have signs or symptoms of a mental impairment or of 
another physical impairment.
    Final 14.00J2 is a new section in this body system. For reasons we 
have already explained, we are removing reference listings--that is, 
listings that are met or equaled by meeting or equaling the criteria of 
another listing--from this body system. However, immune system 
disorders can have effects in virtually every body system, and we 
believe it is important to include guidance about those effects in the 
introductory text so that they are not overlooked.
    Therefore, we are adding section 14.00J2 to explain that immune 
system disorders can have effects in other body systems; we also 
provide a list of examples of those effects in each of the relevant 
body systems with references to other body system listings. These 
provisions are based on language in the second paragraph of prior 
14.00D6, which was relevant only to the evaluation of HIV infection, 
and on the reference listings we are removing. We are expanding the 
information that was in that paragraph to provide specific examples of 
impairments that may be caused by autoimmune disorders.
    For example, prior listings 14.02A6 and 14.04A4 were met with 
evidence of SLE, systemic sclerosis, or scleroderma with ``Digestive 
involvement, as described under the criteria in 5.00ff.'' Apart from 
the fact that these listings were unnecessary because any individual 
who meets the criteria of a listing in the digestive body system (5.00) 
would be disabled under that listing, the guidance was not very 
specific. Also, in the prior rules, we included these criteria only 
under prior listings 14.02 and 14.04. However, other immune system 
disorders can have effects in the digestive system. Therefore, in final 
14.00J2e, we provide that any immune system disorder can have effects 
in the digestive system, and we include an example of hepatitis C in 
addition to providing a reference to 5.00.
    In these final rules, we are adding a reference to weight loss as a 
result of HIV infection that affects the digestive system in final 
14.00J2e. We explain later in this preamble that our reason for adding 
this reference is to respond to public comments we received on the NPRM 
about HIV wasting syndrome.
    Final 14.00J2k provides examples of allergic disorders (including 
skin disorders) that individuals with immune system disorders may have. 
It replaces prior 14.00C.

How are we changing the criteria in the immune system disorders 
listings for adults?

14.01--Category of Impairments, Immune System Disorders

    The following is a detailed explanation of the significant changes 
in the final listings. Some changes are common to several listings, so 
we describe them first.
    1. We are removing all of the reference listings from this body 
system for reasons we have already explained.
    2. We are revising prior listings 14.02B, 14.03B, 14.04B, and 
14.09D (final listings 14.02A, 14.03A, 14.04A, and 14.09B) as follows:
     We are removing the criterion for ``significant, 
documented'' constitutional symptoms or signs in each of these listings 
because we define the constitutional symptoms and signs in final 
14.00C2. Moreover, it is unnecessary to specify ``documented'' because 
we always need to document the existence of any symptom or sign in any 
disability claim.
     Each of these prior listings, except prior listing 14.09D, 
also required you to

[[Page 14582]]

have all four of the constitutional symptoms or signs: Severe fatigue, 
fever, malaise, and involuntary weight loss. We are revising this 
requirement to ``at least two'' of the constitutional symptoms or 
signs, instead of all four, because we believe that the requirement in 
the prior listings was too severe. We believe that any individual with 
an autoimmune disorder involving two or more organs/body systems with 
one organ/body system involved to at least a moderate level of severity 
and who has at least two of the constitutional symptoms and signs in 
these listings will have an impairment that precludes any gainful 
activity. We have also added ``involuntary'' as a descriptor of weight 
loss in final listings 14.02A, 14.03A, 14.04A, 14.05E, 14.06A, 14.07C, 
14.08K, 14.09B, and 14.10A for reasons we explained earlier in this 
preamble.
     In final listings 14.02A, 14.03A, and 14.04A, which 
correspond to prior listings 14.02B, 14.03B, and 14.04B, we are 
removing the reference to ``lesser involvement'' because we are 
removing the prior reference listings to which these rules refer. We 
also believe the phrase is unnecessary--the severity of the impairment 
is demonstrated by the remaining criteria.
    3. As we have already noted under the explanation of final 14.00I, 
we are adding listings based on repeated manifestations accompanied by 
functional limitations and modeled after prior listing 14.08N (final 
listing 14.08K) for each of the other immune system disorders. The 
final listings are:
     14.02B for SLE,
     14.03B for systemic vasculitis,
     14.04D for systemic sclerosis (scleroderma),
     14.05E for polymyositis and dermatomyositis,
     14.06B for undifferentiated and mixed connective tissue 
disease,
     14.07C for immune deficiency disorders, excluding HIV 
infection,
     14.09D for inflammatory arthritis, and
     14.10B for Sj[ouml]gren's syndrome.
    Each listing requires you to have:
     The specified immune system disorder for that listing,
     Repeated manifestations of the specified immune system 
disorder,
     At least two of the constitutional symptoms or signs, and
     A ``marked'' limitation in one of three domains of 
functioning: Activities of daily living, maintaining social 
functioning, or completing tasks in a timely manner due to deficiencies 
in concentration, persistence, or pace.
    We explain what we mean by ``repeated'' in final 14.00I3 and by 
``marked'' in final 14.00I4-5.
    In the final rules, we made a number of changes from the proposed 
rules in response to public comments on the NPRM. Chiefly, we removed 
from several listings the requirement that there must be manifestations 
``without the requisite findings in'' a specified paragraph earlier in 
the listing; for example, proposed listing 14.02B said ``without the 
requisite findings in [14.02]A.'' Our only intent was to explain that 
we would use the listing criterion (for example, listing 14.02B) when 
you have an impairment that does not meet the requirements of the 
previously specified listing section (for example, listing 14.02A). 
However, a public comment pointed out that our language could have been 
confusing, and we determined that it was not necessary to have it at 
all. We explain in detail the public comment and our reasons for making 
this change throughout the final listings in the public comments 
section of this preamble.
    The following is an explanation of the other significant changes we 
are making. We are also making minor editorial changes in some listings 
and changes to cross-references to the introductory text throughout the 
listings to reflect the changes to the introductory text for the final 
rules. We do not describe all of those changes below.

Final Listing 14.04--Systemic Sclerosis (Scleroderma)

    Final listing 14.04B corresponds to prior listing 14.04C. As we 
have already noted, we are expanding this listing to include provisions 
for individuals who had a form of the disorder as children and who 
still have listing-level functional limitations as adults. The final 
listing is essentially identical to final listing 114.04, which we 
describe in detail later in this preamble, except that it includes 
references to appropriate adult rules defining ``inability to ambulate 
effectively'' and ``inability to perform fine and gross movements 
effectively.''
    We are also making minor clarifications in the language of the 
prior listing. Prior listing 14.04C described ``[g]eneralized 
scleroderma with digital contractures.'' We are clarifying that 
``digital'' refers to either the toes or the fingers and are listing 
the effects in the toes separately from the effects in the fingers in 
final listings 14.04B1 and 14.04B2, respectively. We also are removing 
the requirement for ``generalized'' scleroderma (that is, systemic 
sclerosis) because the very serious digital contractures described in 
the final listings would in themselves be disabling regardless of 
whether the scleroderma is generalized.
    Final listing 14.04C corresponds to prior listing 14.04D. We are 
changing ``Raynaud's phenomena'' in prior listing 14.04D to ``Raynaud's 
phenomenon'' for the same reason already described in the explanation 
of final 14.00D3. We are removing the word ``[s]evere'' as a descriptor 
of Raynaud's phenomenon in this listing because it is unnecessary given 
the severity of the impairment demonstrated by the remaining criteria, 
such as ischemia with ulcerations of toes or fingers, resulting in the 
inability to ambulate effectively or to perform fine and gross 
movements effectively. As in final listing 14.04B, we also are 
clarifying that ``digital'' refers to fingers or toes.
    In final listing 14.04C, we are also revising the criteria in prior 
listing 14.04D to provide a better description of listing-level 
Raynaud's phenomenon. The criteria in prior listing 14.04D required 
severe Raynaud's phenomenon characterized by digital ulcerations, 
ischemia, or gangrene. As we noted in the NPRM, we believe that this 
included some individuals who did not have impairments of listing-level 
severity.
    Therefore, in final listing 14.04C1, we provide criteria for 
Raynaud's phenomenon characterized by gangrene involving ``at least two 
extremities'' to establish an impairment that would preclude any 
gainful activity. The final rule is somewhat different from the 
proposed rule, which referred to fingers and toes. We clarified it in 
response to a public comment on the NPRM that we describe in the public 
comments section of this preamble. As in the NPRM, we do not require 
that the gangrene result in the inability to ambulate effectively or to 
perform fine and gross movements effectively because the presence of 
gangrene involving at least two extremities by itself demonstrates a 
very serious impairment.
    In final listing 14.04C2, we provide criteria for ischemia with 
ulcerations of the toes or fingers that results in the inability to 
ambulate effectively or to perform fine and gross movements 
effectively; Raynaud's phenomenon characterized only by ischemia with 
ulcerations does not, by itself, describe an impairment that would 
necessarily result in an extreme loss of function. Also, ulcerations 
are an outcome of ischemia, so we are revising the language of the 
prior rule so that ischemia and ulcerations are not listed as though 
they are separate entities.

[[Page 14583]]

Final Listing 14.05--Polymyositis and Dermatomyositis

    Final listing 14.05A corresponds to prior listing 14.05A. We are 
replacing the word ``severe'' as a descriptor of proximal limb-girdle 
weakness with the more accurate ``resulting in inability to ambulate 
effectively or inability to perform fine and gross movements 
effectively, as defined in 14.00C6 and 14.00C7.'' We are also changing 
``shoulder and/or pelvic'' muscle weakness to ``pelvic or shoulder'' 
muscle weakness because either pelvic muscle weakness that results in 
the inability to ambulate effectively or shoulder muscle weakness that 
results in the inability to perform fine and gross movements 
effectively is sufficient in itself to show disability, and the ``and'' 
is unnecessary.
    Final listing 14.05B corresponds to prior listing 14.05B1. We are 
removing a number of the requirements from the prior rule because we 
have determined that impaired swallowing with aspiration due to muscle 
weakness establishes a listing-level impairment. We are revising the 
requirement for ``episodes of aspiration'' to only ``aspiration'' 
because of the progressive nature of muscle weakness that results from 
polymyositis or dermatomyositis. Once an episode of aspiration is 
documented, further documentation of multiple episodes is unnecessary. 
In addition, we are replacing ``cricopharyngeal weakness'' with 
``muscle weakness'' in final 14.05B because impaired swallowing and 
aspiration may result from muscles other than the cricopharyngeal 
muscles. Finally, we are revising the phrase ``impaired swallowing with 
dysphagia'' to ``impaired swallowing (dysphagia)'' because 
``dysphagia'' means impaired swallowing.
    Final listing 14.05C corresponds to prior listing 14.05B2, for 
individuals who have polymyositis or dermatomyositis with impaired 
respiration due to intercostal and diaphragmatic muscle weakness.
    Final listing 14.05D, Diffuse calcinosis, is a new listing for 
adults that has the same criteria as final listing 114.05D for 
children, which we describe in detail later in this preamble. We are 
adding this listing for individuals who had a form of the disorder as 
children and who still have listing-level functional limitations as 
adults.

Final Listing 14.06--Undifferentiated and Mixed Connective Tissue 
Disease

    We are changing the heading of prior 14.06 to update it and to more 
accurately describe the disorders we evaluate under this listing.
    Prior listing 14.06 was entirely a reference listing, requiring 
evaluation under prior listings 14.02A, 14.02B, or 14.04. We are 
changing it to a stand-alone listing. Final listing 14.06A contains the 
same criteria as final listings 14.02A, 14.03A, and 14.04A; that is, 
involvement of two or more body systems to at least a moderate level of 
severity and at least two of the constitutional symptoms or signs. 
Final listing 14.06B contains the same functional criteria for the 
evaluation of repeated manifestations of undifferentiated and mixed 
connective tissue disease as the other listings in this body system.

Final Listing 14.07--Immune Deficiency Disorders, Excluding HIV 
Infection

    We are changing the heading of listing 14.07 to update its 
terminology and to more accurately describe the disorders we evaluate 
under this listing.
    The prior listing was met with documented, recurrent severe 
infections occurring three or more times within a 5-month period. We 
are replacing this criterion with a more accurate and up-to-date 
listing. The listing is in three parts.
    Final listing 14.07A is essentially the same as final listing 
14.08J (prior listing 14.08M), which describes individuals with HIV 
infection whose immune systems are so compromised that they frequently 
become ill. We believe that these criteria for individuals with HIV 
infection are equally as applicable to individuals with other kinds of 
immune deficiency disorders, and that they are more inclusive than the 
criteria in prior listing 14.07.
    As in final listing 14.08J, final listing 14.07A provides that the 
infections must occur three times in a 12-month period, not three times 
in only a 5-month period. It also more precisely explains how severe 
the infections need to be by requiring either resistance to treatment 
or a need for hospitalization or intravenous treatment. It also 
specifies six types of infections.
    Final listing 14.07B is new. We are adding this listing to 
recognize that some immune system disorders are treated by stem cell 
transplantation. In final listing 14.07B, we state that we will 
consider you to be under a disability until at least 12 months from the 
date of transplantation and, thereafter, evaluate any residual 
impairment(s) under the criteria for the affected body system.
    Final listing 14.07C incorporates the same functional criteria for 
the evaluation of repeated manifestations of immune deficiency 
disorders (excluding HIV infection) as in the other final listings in 
this body system and for the same reasons as described above.

Final Listing 14.08--Human Immunodeficiency Virus (HIV) Infection

    Except as described below, we are not making any changes to the 
criteria in listing 14.08. As noted in the NPRM, we carefully 
considered the advances in treatment and consequent increases in 
longevity that have occurred since we published the prior rules in 
1993. Based on this review, we did not believe that there had been 
sufficient progress in the treatment and control of HIV infection to 
warrant any change in these rules at that time. However, as a result of 
public comments we received on the NPRM, we now believe that some 
changes may be appropriate. Therefore, while final listing 14.08 is 
substantively the same as proposed listing 14.08, we are publishing 
separately an ANPRM in today's edition of the Federal Register inviting 
comments and suggestions on how to update and revise our listing for 
HIV infection. We will consider the comments and suggestions that we 
receive in response to the ANPRM, as well as our adjudicative 
experience and additional information about advances in medical 
knowledge, treatment, and methods of evaluating HIV infection. If we 
determine that listing 14.08 should be revised, we will publish for 
public comment an NPRM that will propose specific revisions to the 
listing.
    As already noted, we are removing reference listings throughout 
this body system, including the reference listings in listing 14.08. 
This results in the removal of several specific listings within 14.08 
and the redesignation of some of the prior listings; for example, prior 
listing 14.08N has become final listing 14.08K. Where we are removing a 
reference listing, however, we have ensured that we provide guidance in 
the introductory text about where to evaluate the impairment. For 
example, prior listing 14.08A4, for HIV infection with syphilis or 
neurosyphilis, was a reference listing that said only to consider the 
impairment under the criteria for the affected body system, such as 
2.00 (special senses and speech), 4.00 (cardiovascular system), or 
11.00 (neurological). Although we are removing this reference listing, 
we include this same guidance in final 14.00J2l.
    We are also clarifying some of the rules. In final listing 14.08B2, 
we are reorganizing the language from prior listing 14.08B2 to make it 
clearer that we evaluate under this listing candidiasis involving the 
esophagus,

[[Page 14584]]

trachea, bronchi, or lungs, or at another site other than the skin, 
urinary tract, intestinal tract, or oral or vulvovaginal mucous 
membranes. We are moving prior listing 14.08C2, for PCP, from the 
listing for protozoan and helminthic infections to the listing for 
fungal infections because the organism that causes PCP is now known to 
be a fungus. We redesignate it as final listing 14.08B7.
    We are redesignating prior listing 14.08N as final listing 14.08K. 
We are expanding our guidance on manifestations we evaluate under final 
listing 14.08K by adding ``pancreatitis, hepatitis, peripheral 
neuropathy, glucose intolerance, muscle weakness, cognitive or other 
mental limitation'' as new examples. We are also expanding our list of 
signs and symptoms by adding ``nausea, vomiting, headaches, or 
insomnia.''
    We made minor changes to the language of the functional criteria in 
final listing 14.08K from the language in prior listing 14.08N. For 
example, we replaced the words ``restriction'' in prior listing 14.08N1 
and ``difficulties'' in prior listings 14.08N2 and 14.08N3 with the 
word ``limitation'' in final listings 14.08K1, 14.08K2, and 14.08K3. We 
made this change because ``limitation'' is a more accurate description 
for the functional criteria in these listings.
    We are making a number of changes from the proposed rule in 
response to public comments on the NPRM and for editorial reasons. The 
changes are in:
     Final listing 14.08B2, in which we made a minor editorial 
correction to remove a redundant word;
     Final listing 14.08B7, in which we removed the word 
``carinii'' and the parenthetical ``jiroveci'' from the name of 
``Pneumocystis pneumonia'' in response to a public comment on the NPRM;
     Final listing 14.08E4, in which we revised the criterion 
from ``squamous cell carcinoma of the anus'' to ``squamous cell 
carcinoma of the anal canal or anal margin'' in response to a public 
comment on the NPRM; \1\
---------------------------------------------------------------------------

    \1\ We also made minor conforming changes in prior 13.00A and 
113.00A of the malignant neoplastic diseases listings to reflect 
this change.
---------------------------------------------------------------------------

     Final listing 14.08H, in which we clarified that the 10 
percent loss of weight from baseline may be calculated in pounds, 
kilograms, or by body mass index (BMI) in response to a public comment 
on the NPRM;
     Final listing 14.08J, in which we removed an unnecessary 
comma; and
     Final listing 14.08K, in which we changed the reference to 
``fatigue'' to ``severe fatigue'' and a reference to a ``mental 
impairment'' to a ``mental limitation'' in response to public comments 
on the NPRM, and removed the proposed cross-reference to 14.00I5. The 
removal of the cross-reference is only editorial. The reference was 
unnecessary, incomplete (the term ``marked'' for the various domains is 
also defined in final 14.00I6, 14.00I7, and 14.00I8), and inconsistent 
with other sections of the proposed immune disorder listings which 
contained the same severity criteria but did not include this cross-
reference.
    We provide detailed explanations of the changes we made in response 
to public comments on the NPRM and our reasons for making them in the 
public comments section of this preamble.

Final Listing 14.09--Inflammatory Arthritis

    We are redesignating prior listing 14.09D as final listing 14.09B, 
prior listing 14.09B as final listing 14.09C1, and prior listing 14.09E 
as final listing 14.09C2 to put these listings in a more logical order. 
In the final rules, listing 14.09A describes persistent inflammation or 
deformity of major peripheral joints that alone is disabling, while 
listing 14.09B describes disability with lesser inflammation or 
deformity of major peripheral joints together with organ involvement 
and constitutional symptoms or signs. Final listing 14.09C describes 
listing-level inflammatory arthritis of the spine. Final listing 
14.09C1 describes disability based only on fixation (ankylosis) of the 
spine, while final listing 14.09C2 describes disability based on a 
lesser degree of ankylosis of the spine with organ involvement. Final 
listing 14.09D is the same functional listing we include in all of the 
final immune system disorders listings and applies to inflammatory 
arthritis affecting any joints.
    Final listing 14.09A corresponds to prior listing 14.09A. We are 
removing the requirement for a history of joint pain, swelling, and 
tenderness from this listing because it is unnecessary. (We do refer to 
joint pain, swelling, and tenderness in final 14.00D6a as possible 
signs and symptoms of the disorder.) Persistent joint inflammation or 
deformity in one or more major peripheral weight-bearing joints 
resulting in the inability to ambulate effectively, or persistent joint 
inflammation or deformity of major peripheral joints in both upper 
extremities resulting in inability to perform fine and gross movements 
effectively, is in itself indicative of an impairment that would 
preclude any gainful activity. For the same reasons, we are also 
removing the requirement for ``signs on current physical examination.'' 
We do not need signs of joint inflammation on a current physical 
examination when we have medical evidence documenting that you have 
inflammatory arthritis that results in the inability to ambulate 
effectively or inability to perform fine and gross movements 
effectively. Also, because of the episodic nature of inflammatory 
arthritis, a current physical examination could show a brief period of 
improvement for a few days even though your longitudinal medical 
records may show persistent joint inflammation that results in the 
inability to ambulate effectively or inability to perform fine and 
gross movements effectively.
    As we noted under the explanation of final 14.00D6e, we are 
revising listing 14.09A in response to a public comment on the NPRM so 
that there is no longer a need to use listing 1.02 or 1.03 in cases 
involving inflammatory arthritis. Final listing 14.09 (and final 
listing 114.09) will apply to all individuals who have listing-level 
limitations as a result of inflammatory arthritis. The revised listing 
includes essentially the same requirements as listings 1.02 and 1.03 of 
the musculoskeletal listings.
    Because of this, we are changing the structure of final listing 
14.09A to provide separate criteria for inflammatory arthritis that 
involves one or more major peripheral weight-bearing joints (final 
listing 14.09A1) and inflammatory arthritis involving one or more major 
peripheral joints in both upper extremities (final listing 14.09A2), 
with appropriate severity criteria for each. We define the ``major 
peripheral joints'' in final 14.00C8.
    Final listing 14.09B corresponds to prior listing 14.09D. The 
revisions in final 14.09B are similar to those in final listing 14.09A 
for the same reasons and to make it clearer that this listing requires 
joint inflammation in one or more major peripheral joints. Final 14.09B 
continues to require less joint involvement than in A, but we no longer 
require ``lesser extra-articular features than in C'' because ``C'' 
refers to prior reference listing 14.09C, which we have removed. Final 
listing 14.09B1 corresponds to prior listing 14.09D2 with 
nonsubstantive editorial changes to make it consistent with how we 
present this criterion throughout these listings. Final listing 14.09B2 
corresponds to prior listing 14.09D1 except that we have removed the 
phrase ``significant, documented'' for reasons we have already 
explained. We are also correcting an error in prior listing 14.09D1. 
The explanatory abbreviation, ``e.g.'' (for example) in prior listing

[[Page 14585]]

14.09D1 inaccurately indicated that the four constitutional symptoms or 
signs, that is, severe fatigue, fever, malaise, and involuntary weight 
loss, were only examples when they are in fact a complete list. 
Consistent with changes in other final listings, we are requiring at 
least two of the constitutional symptoms or signs because we believe 
that the criteria in final listing 14.09B are indicative of an 
impairment that precludes any gainful activity.
    Final listing 14.09C1 corresponds to prior listing 14.09B. We are 
reorganizing the criteria and removing the requirements for ``diagnosis 
established by findings of unilateral or bilateral sacroiliitis (e.g., 
erosions or fusions)'' and ``[h]istory of back pain, tenderness, and 
stiffness'' because these findings are unnecessary. We believe 
ankylosing spondylitis or other spondyloarthropathies with ankylosis of 
the dorsolumbar or cervical spines at 45[deg] or more of flexion 
documented as required in final listing 14.09C1 are in themselves 
indicative of an impairment that precludes any gainful activity.
    Final listing 14.09C2 corresponds to prior listing 14.09E. We are 
reorganizing this listing to make it more consistent with the structure 
and criteria that we use in the final listings for other autoimmune 
disorders. We are removing the phrase ``with lesser deformity than in 
B,'' which describes a deformity that is less than the fixation ``of 
the dorsolumbar or cervical spine at 45[deg] or more of flexion'' under 
prior listing 14.09B, and replacing it with fixation ``at 30[deg] or 
more of flexion (but less than 45[deg]).'' We believe that this is a 
clearer and more specific criterion that helps to provide greater 
uniformity in adjudications under this listing. We are removing the 
phrase ``lesser extra-articular features than in C'' because it refers 
to prior reference listing 14.09C, which we are removing. We also are 
removing the phrase ``with signs of unilateral or bilateral 
sacroiliitis'' because the criteria in the final listing would be 
sufficient to show listing-level severity without this requirement, and 
the phrase ``with the extra-articular features described in 14.09D'' 
because it is unnecessary.

Final Listing 14.10--Sj[ouml]gren's Syndrome

    Final listing 14.10 is new. We are adding it in response to 
comments we received before we developed the NPRM indicating that 
Sj[ouml]gren's syndrome is distinct from other immune system disorders 
and that it has unique aspects that the prior immune system listings 
did not address.
    Although individuals with Sj[ouml]gren's syndrome were able to 
qualify under prior listings 14.03 and 14.09 and other listings, we 
believe that it is now appropriate to list Sj[ouml]gren's syndrome 
separately in these listings. We are using the same two listing 
criteria for establishing listing-level severity as in the other final 
listings for autoimmune disorders because Sj[ouml]gren's syndrome is an 
autoimmune disorder that can cause the same kinds of constitutional 
symptoms and signs as other autoimmune disorders, and because it can be 
as functionally limiting as other autoimmune disorders. Final listing 
14.10A is the same as final listings 14.02A, 14.03A, 14.04A, and 
14.06A, and final listing 14.10B is the same as final listings 14.02B, 
14.03B, 14.04D, 14.05E, 14.06B, and 14.09D. As already noted, we also 
provide a new separate section in the introductory text that describes 
the unique features of Sj[ouml]gren's syndrome, final 14.00D7.

How are we changing the introductory text for the immune system 
disorders listings for children?

    As in final 14.00 in the adult rules, we are changing the name of 
this body system to ``Immune System Disorders.''
    Except for minor editorial changes, we have repeated much of the 
introductory text of final 14.00 in the introductory text of final 
114.00. This is because the same basic rules for establishing and 
evaluating the existence and severity of immune system disorders in 
adults also apply to children. Because we have already described these 
provisions under the explanation of final 14.00, the following 
discussions describe only those provisions that are unique to the 
childhood rules or that require further explanation. We describe only 
the major provisions. For example, we do not summarize minor editorial 
changes that refer to ``children'' instead of adults or to the policy 
of ``functional equivalence'' instead of RFC assessment and steps in 
the adult sequential evaluation process.
    Also, where appropriate in the introductory text of final 114.00, 
we have made an editorial change from the prior rules in the terms we 
use to identify the age categories of children in the introductory text 
of prior 114.00 to be consistent with the terms we use in the 
introductory text of current 112.00, Mental disorders. For example, in 
final 114.00F1b(ii), we use ``newborn and younger infants (birth to 
attainment of age 1)'' instead of ``an infant 12 months of age or 
less'' as in prior 114.00D3b(i).
    Finally, we have changed the part B final rules from the NPRM in 
the same way that we changed the part A final rules from the NPRM 
whenever those proposed rules were the same.

Final 114.00A--What disorders do we evaluate under the immune system 
disorders listings?

    In final 114.00A1b, we incorporate the first sentence in the last 
paragraph of prior 114.00B, which explains that immune system disorders 
may affect growth, development, attainment of age-appropriate skills, 
and performance of age-appropriate activities in children. We are 
revising the sentence by adding the phrase ``or their treatment.'' We 
are also removing the phrase ``attainment of age-appropriate skills'' 
because it is redundant of ``development.''
    Final 114.00A2 is essentially the same as final 14.00A2 and similar 
to the first and second paragraphs of prior 114.00B. We are expanding 
and clarifying the guidance in the second paragraph to explain that 
autoimmune disorders or their treatment may have a considerable impact 
on the physical, psychological, and developmental growth of pre-
pubertal children that often differs from that of post-pubertal 
children or adults. We are also removing the last sentences from both 
the first and second paragraphs of prior 114.00B because they cross-
referred to 14.00 in the part A listings. In part B of these final 
rules, we are repeating criteria from part A when they are appropriate 
for evaluating children so it should rarely be necessary to refer back 
to 14.00 in part A.

Final 114.00D--How do we document and evaluate the listed autoimmune 
disorders?

    Final 114.00D parallels the structure and content of final 14.00D 
in the adult rules, except where the features commonly associated with 
the autoimmune disorders in these listings differ in children from 
adults.
    In final 114.00D2, Systemic vasculitis (114.03), as in prior 
114.00C3, we provide guidance (in final 114.00D2a(ii)) on how we 
evaluate Kawasaki disease and add guidance about anaphylactoid purpura 
(Henoch-Schoenlein purpura). Also, in final 114.00D2a(ii), we do not 
use the example of giant cell arteritis (temporal arteritis) that is in 
final 14.00D2a(ii) because this disorder occurs almost exclusively in 
individuals over 50 years of age.
    In final 114.00D3c, Localized scleroderma (linear scleroderma or 
morphea), we describe features of focal forms of scleroderma in 
children. These disorders occur primarily in children and are more 
common than systemic sclerosis in children. In final

[[Page 14586]]

114.00D3c(i), we explain that the extent of involvement and the 
location of the lesions are important factors in determining the 
limitations resulting from scleroderma. We also note that it may be 
appropriate to evaluate the limitations resulting from these 
impairments under the musculoskeletal listings (101.00).
    In final 114.00D3c(ii), we describe features of isolated morphea of 
the face and explain that it may be more appropriate to evaluate the 
limitations from these disorders under the affected body system, such 
as special senses and speech (102.00) or mental disorders (112.00). We 
have made a minor correction in the final rule. In the NPRM, we 
indicated that it would be more appropriate to evaluate the limitations 
from these disorders only under the special senses or mental disorders 
listings. However, we explained in the preamble that these body systems 
were only examples of body systems that might be affected. In the final 
rule, we are clarifying that the body systems we cite are only 
examples. We have made the same correction in part A.
    In final 114.00D3c(iii), we describe musculoskeletal and 
respiratory features of chronic variants of these syndromes and explain 
that it is appropriate to evaluate the limitations from these disorders 
under the musculoskeletal listings (101.00) or respiratory system 
listings (103.00).
    In final 114.00D4, Polymyositis and dermatomyositis (114.05), we 
note (in final 114.00D4a, General) that polymyositis occurs rarely in 
children and describe the features of dermatomyositis that occur 
differently in children than in adults.
    In children, polymyositis and dermatomyositis usually do not occur 
in association with malignancies. For this reason, we do not include a 
reference to malignancy or provide guidance that we will evaluate 
malignancies under the malignant neoplastic diseases listings (113.00) 
in final 114.00D4, as we do for adults in final 14.00D4. However, 
unlike in the adult rules, we include a reference to calcinosis for 
children in this section. Calcinosis is primarily an outcome of 
juvenile dermatomyositis; when adults with dermatomyositis have 
calcinosis, it is generally because they have had the condition since 
childhood. For this reason, we refer to calcinosis only in the 
introductory text for children, final 114.00D4. However, we include a 
criterion for diffuse calcinosis in final listing 14.05D (as well as 
final listing 114.05D) for adults who have the condition. Also, when 
dermatomyositis involves other organs or body systems, we evaluate the 
involvement under the affected body system.
    In final 114.00D4b, Documentation of polymyositis or 
dermatomyositis, we note that magnetic resonance imaging (MRI) showing 
muscle inflammation or vasculitis provides additional evidence of 
childhood dermatomyositis. We did not provide this guidance in final 
14.00D4b because MRI findings are not considered diagnostic of 
dermatomyositis in adults. Similar to final 14.00D4b, we added two 
sentences to the final rule to indicate that when the results of 
electromyography, muscle biopsy, or MRI are in your medical records we 
will make every reasonable effort to obtain them, but that we will not 
purchase any of these tests.
    In final 114.00D4c(i), we explain how to evaluate polymyositis and 
dermatomyositis under the listings in newborn and younger infants.
    In final 114.00D5, Undifferentiated and mixed connective tissue 
disease (114.06), we note (in final 114.00D5a, General) that the most 
common pattern of undifferentiated autoimmune disorders in children is 
mixed connective tissue disease (MCTD). In final 114.00D5b, 
Documentation of undifferentiated and mixed connective disease, we note 
diagnostic laboratory findings specifically for children with MCTD and 
that the clinical findings are often suggestive of SLE or childhood 
dermatomyositis. We also note that many children later develop features 
of scleroderma.
    In final 114.00D6, Inflammatory arthritis (114.09), we incorporate 
(in final 114.00D6a, General) guidance from prior 114.00C2 and 114.00E. 
We explain that we evaluate growth impairment resulting from 
inflammatory arthritis under the criteria in 100.00. In final 
114.00D6b, Inflammatory arthritis involving the axial spine 
(spondyloarthropathy), we incorporate the second sentence in prior 
114.00E and revise some of the examples of disorders that may be 
associated with inflammatory spondyloarthropathies involving the axial 
spine with disorders that are more common in children.
    Prior 114.00E6 provided that the fact that a child is dependent on 
steroids, or any other drug, for the control of inflammatory arthritis 
is, in and of itself, insufficient to find disability. It explained 
that advances in the treatment of inflammatory connective tissue 
disease and in the administration of steroids for its treatment have 
corrected some of the previously disabling consequences of continuous 
steroid use. Although this statement is still true, we are not 
including this provision of prior 114.00E6 in these final rules because 
we believe we no longer need it in the introductory text for the 
listings.
    We added prior 114.00E6 in 2002 (66 FR at 58022 and 58045). It was 
important when we added it because the listings prior to the revisions 
we made in 2002 included a listing (prior listing 101.02B) that said 
that all children with rheumatoid arthritis who were dependent on 
steroids were disabled. We removed that listing in 2002, explaining 
that, although the prior listing was appropriate when we first 
published it, advances in treatment and other reasons had made it 
obsolete (66 FR at 58022). Thus, the paragraph in the introductory text 
served as a reminder that we no longer had that listing and that it was 
no longer appropriate to presume disability based on steroid use alone. 
Now that several years have passed since we removed the prior listing, 
we do not believe that we need this reminder any longer. However, in 
final 114.00G3, we continue to state that we will consider the adverse 
side effects of treatment, including the effects of corticosteroids, to 
ensure that our adjudicators remember to consider the side effects of 
steroids and any other treatment an individual might have.

Final 114.00F--How do we document and evaluate human immunodeficiency 
virus (HIV) infection?

    Final 114.00F parallels the structure and content of final 14.00F 
in the adult rules, except where the features commonly associated with 
HIV infection differ in children from adults.
    Final 114.00F1a, Definitive documentation of HIV infection, 
corresponds to 114.00D3a in the prior rules and 14.00F1a in the final 
rules. In final 114.00F1a(i), we are lowering the age for using HIV 
antibody tests from the 24 months of age or older that was in prior 
114.00D3a(i) to 18 months or older. Current clinical practice now 
accepts these tests beginning at 18 months of age.
    In final 114.00F1a(iv), we clarify the provision in prior 
114.00D3a(ii) by explaining that a specimen that contains HIV antigen 
may be used to establish the diagnosis of HIV infection in a child age 
1 month or older.
    Final 114.00F1b, Definitive documentation of HIV infection in 
children from birth to the attainment of 18 months, corresponds to the 
second paragraph in prior 114.00D3b, Other acceptable documentation of 
HIV infection in children. We are moving this information and revising 
the age cutoff to 18 months to recognize that laboratory values we 
previously considered to be ``other acceptable

[[Page 14587]]

documentation'' of HIV infection are now considered definitively 
diagnostic in children from birth to age 18 months who have tested 
positive for HIV antibodies.
    In final 114.00F1b(i), we add ``One or more of the tests listed in 
F1a(ii)-F1a(vii)'' of final 114.00F1a because these tests are accepted 
as diagnostic of HIV infection.
    In final 114.00F1b(iii), we change ``12 to 24 months of age'' in 
current 114.00D3b(ii) to ``12 to 18 months of age'' based on how these 
findings are used in current clinical practice.
    In final 114.00F1b(v), we specify that a severely diminished 
immunoglobulin G (IgG) level is ``< 4g/l or 400 mg/dl.'' However, we do 
not provide an IgG level for greater than normal range for age due to 
the variability in the higher normal range of IgG level in children by 
age. There is consistency in the normal lower average range in 
children, so we are able to specify levels for severely diminished IgG.
    Final 114.00F1c, Other acceptable documentation of HIV infection, 
corresponds to prior 114.00D3b and final 14.00F1b. We are removing the 
first paragraph in prior 114.00D3b, which explained that HIV infection 
is not documented in children under 24 months of age by a serum 
specimen containing HIV antibodies. All infants who have HIV antibodies 
are now tested to determine definitively whether they have HIV 
infection.
    In final 114.00F2, CD4 tests, we add more detailed guidance to the 
second paragraph of prior 114.00D4a by specifying that the extent of 
immune depression correlates with the level of CD4 counts (relative to 
the age of the child), and that by age 6, CD4 levels become comparable 
to adult CD4 levels.
    In final 114.00F3b, Other acceptable documentation of the 
manifestations of HIV infection, we explain, in 114.00F3b(i) for PCP 
and in 114.00F3b(ii) for CMV disease, that a CD4 count below 200 in 
children 6 years of age or older is supportive evidence of a 
presumptive diagnosis of these manifestations.
    Final 114.00F4, HIV manifestations specific to children, 
corresponds to prior 114.00D5, HIV in children. In final 114.00F4a, 
General, we are removing the second sentence in prior 114.00D5. That 
sentence explained that survival times were shorter for children who 
were infected in the first year of life than they were for older 
children and adults. However, due to advances in medical treatment this 
is no longer the case. The second sentence of final 114.00F4a is based 
on the first paragraph in prior 114.00D5.
    In final 114.00F4b, Neurologic abnormalities, we make some 
nonsubstantive editorial changes to the second paragraph in prior 
114.00D5 in which we explained that the methods of identifying and 
evaluating neurological abnormalities vary depending on a child's age. 
We also replace ``acquisition'' with ``onset'' in the last sentence of 
final 114.00F4b because a sudden ``onset'' of a new learning disability 
is medically a more accurate description of how this neurologic 
abnormality would manifest in a child with HIV infection.
    In final 114.00F4c, Bacterial infections, we incorporate the last 
two paragraphs in prior 114.00D5. We make only nonsubstantive editorial 
changes, including removing text that only repeats criteria from the 
listings.

Final 114.00G--How do we consider the effects of treatment in 
evaluating your autoimmune disorder, immune deficiency disorder, or HIV 
infection?

    In final 114.00G2, Variability of your response to treatment, we 
use an example of a child who develops otitis media instead of 
pneumonia or tuberculosis as we do in final 14.00G2 for an adult 
because otitis media is more common in children.
    In final 114.00G3, How we evaluate the effects of treatment for 
autoimmune disorders on your ability to function, we use examples of 
impaired growth and osteopenia instead of osteoporosis as we do in 
final 14.00G3 because impaired growth and osteopenia are more common in 
children.

Final 114.00I--How do we use the functional criteria in these listings?

    As in the adult rules, we are adding listings based on functional 
criteria to each of the listings in the immune system in addition to 
those that are already in listing 114.08. Final 114.00I--How do we use 
the functional criteria in these listings?--corresponds to prior 
114.00D8 and provides guidance for applying the listings based on 
functional criteria in all of the final childhood listings. We revised 
the prior language to reflect the fact that there are now functional 
listings for each of the listed impairments in this body system and for 
consistency with adult rules where appropriate.

Final 114.00J-- How do we evaluate your immune system disorder when it 
does not meet one of these listings?

    In final 114.00J2, we repeat the guidance in final 14.00J but with 
appropriate references to childhood listings in part B, including an 
example of growth impairment under 100.00.

How are we changing the criteria in the immune system disorders 
listings for children?

Final 114.01--Category of Impairments, Immune System Disorders

    As in the adult listings in part A, we are removing all reference 
listings from part B. We also add listings like final listing 114.08L 
(prior listing 114.08O) for each of the other listed impairments in 
this body system. (As in the NPRM, we are redesignating prior listing 
114.08O as final listing 114.08L because of the deletion of reference 
listings.) The new listings are final listings 114.02B, 114.03B, 
114.04D, 114.05E, 114.06B, 114.07C, 114.09D, and 114.10B. The 
functional criteria in the final listings for children are the same as 
in prior listing 114.08O, using the functional criteria in listings 
112.02 and 112.12. They are different from the functional criteria in 
part A because the childhood functional criteria vary depending on the 
age of the child and are a better way to measure broad functional 
limitations in children.
    The following is a description of the significant changes in part B 
when they are different from the changes we made in part A or require 
additional explanation.

Final Listing 114.04--Systemic Sclerosis (Scleroderma)

    Final listings 114.04B1 and 114.04B2 correspond to prior listing 
114.04B1. We are changing the requirement in prior listing 114.04B1 for 
fixed deformity of ``both feet'' to ``one or both feet'' and adding 
``inability to ambulate effectively'' to the listing criteria. This 
will allow some children with a serious deformity in only one foot to 
qualify based on the functional limitation we use to define listing-
level severity throughout these listings. We are also adding a 
criterion for ``toe contractures'' to final 114.04B1, even though toe 
contractures of listing-level severity would be rare in children, to 
make it consistent with the criterion in final 14.04B1. We are 
retaining the requirement for involvement of both hands in final 
listing 114.04B2, because inability to perform fine and gross movements 
effectively can occur only when both upper extremities are affected. We 
are adding the criterion of ``finger contractures'' to final 114.004B2 
for the same reason we are adding ``toe contractures'' to final 
114.04B1.
    Final listings 114.04B3 and 114.04B4 correspond to prior listing 
114.04B2, the listing for ``[m]arked destruction or marked atrophy of 
an extremity.'' We are revising the prior rules to:
     Remove the word ``marked,''

[[Page 14588]]

     Change the criterion for ``destruction'' to ``irreversible 
damage,''
     Require both atrophy and irreversible damage in one or 
both lower extremities or both upper extremities, and
     Require either inability to ambulate effectively or to 
perform fine and gross movements effectively.
    We are removing the word ``marked'' because we use it in various 
other listings and other regulations to describe a particular measure 
of functional limitations, and it does not describe what we intend in 
this listing. We are replacing the criterion for ``marked destruction'' 
with a criterion for ``irreversible damage'' because it is a more 
accurate medical description of this complication of systemic 
sclerosis. We are requiring both atrophy and irreversible damage 
because we would not expect either of these findings alone to establish 
an impairment that results in marked and severe functional limitations 
in every case. Finally, we are requiring ``inability to ambulate 
effectively'' or ``inability to perform fine or gross movements 
effectively'' to establish an impairment that is of listing-level 
severity, consistent with other listings.
    Final listing 114.04C, Raynaud's phenomenon, is a new childhood 
listing and has the same criteria as in final listing 14.04C for 
adults.

Final Listing 114.05--Polymyositis and Dermatomyositis

    We are removing prior listing 114.05B1 because multiple joint 
contractures are not typically a part of the disease process of 
polymyositis or dermatomyositis in children. However, if this should 
occur, we would evaluate whether your polymyositis or dermatomyositis 
with multiple joint contractures meets or medically equals the criteria 
in final listing 114.05E, medically equals the criteria in another 
listing, such as final listing 114.05A, or functionally equals the 
listings.
    In final listing 114.05D, we are revising prior listing 114.05B2 by 
replacing ``cutaneous calcification'' with ``calcinosis.'' We are 
making this change because ``calcification'' describes the normal 
process by which calcium salts are deposited in bone, and 
``calcinosis'' describes the abnormal deposits of calcium salt in body 
tissues as we intend by this criterion. We are also replacing 
``formation of an exoskeleton'' with ``limitation of joint mobility or 
intestinal motility'' because it is a better description of the known 
complications of dermatomyositis in children.

Final Listing 114.07--Immune Deficiency Disorders, Excluding HIV 
Infection

    We are removing prior listing 114.07B because of advances in 
medical knowledge that now allow the identification of different 
subgroups of thymic dysplastic syndromes. The subgroups of these 
disorders vary in severity, and therefore, we will evaluate them under 
final listing 114.07A, B, or C, as appropriate to the particular immune 
deficiency disorder and its effects.

Final Listing 114.08--Human Immunodeficiency Virus (HIV) Infection

    In final listing 114.08A4, we have added a reference to final 
114.00F4c in response to a public comment on the NPRM about children 
who are age 13 or older, whose impairments cannot meet but can 
medically equal this listing. In final listing 114.08A5, we incorporate 
prior listing 114.08A6 except to remove ``Other'' as a descriptor to 
make it consistent with the final adult listing. We replace 
``acquisition'' as used in prior listing 114.08H1 with ``onset'' in 
final listing 114.08G1 because a sudden ``onset'' of a new learning 
disability is medically a more accurate description of how this 
neurologic abnormality would manifest in a child with HIV infection. We 
are also redesignating a number of listings to reflect the removal of 
reference listings.

Final Listing 114.10-- Sj[ouml]gren's Syndrome

    We are adding listing 114.10 to evaluate Sj[ouml]gren's syndrome in 
children for the same reasons we are adding a Sj[ouml]gren's syndrome 
listing for adults in part A.

Other Changes

    We are making minor conforming changes in prior 1.00B and 101.00B, 
and 1.00L and 101.00L to reflect changes in the final immune body 
system listings.
    We are also making minor conforming changes in prior 8.00D3 and 
108.00D3 of the skin disorders listings. We are revising these sections 
to indicate that we evaluate Sj[ouml]gren's syndrome under the new 
listing for that disorder, final listings 14.10 and 114.10.
    We are also making minor conforming changes in prior 13.00A and 
113.00A of the malignant neoplastic diseases listings. We are revising 
these sections to reflect changes in final listings 14.08E and 114.08E.
    Throughout these final rules, we are also making a number of minor 
editorial changes from the NPRM that we have not summarized above. For 
example, we have corrected unintentional language inconsistencies 
between part A and part B, changed sentences to use active voice 
instead of passive voice, and removed some repetitive statements and 
unnecessary words. None of these revisions are substantive, and they do 
not change the meaning of what we originally proposed in the NPRM.

Public Comments on the NPRM

    In the NPRM, we published in the Federal Register on August 04, 
2006 (71 FR 44432, corrected at 71 FR 46983), we provided the public 
with a 60-day comment period that ended on October 13, 2006. In 
addition to our notice to the public, we invited comments from national 
medical organizations and professionals, advocacy groups, and legal 
services organizations.
    We received 55 comment letters. The commenters included advocacy 
groups, legal services organizations, State agencies that make 
disability determinations for us, medical organizations, and 
individuals, including individuals who have immune system disorders or 
relatives with immune system disorders. One of the comment letters 
reflected the comments from 40 organizations. We carefully considered 
all of the comments and provide our reasons for adopting or not 
adopting the comments in our responses below. Because some of the 
comments were long, we have condensed, summarized, and paraphrased 
them. We believe we have presented the commenters' views accurately, 
and have responded to all of the significant issues raised by the 
commenters that were within the scope of these rules.
    Some commenters also wrote in about issues that were not related to 
the proposed rules, and in some cases not to Social Security disability 
benefits. Although we did read those letters, we did not respond to 
them.
    Also, some commenters sent comments supporting the rules changes 
and noting provisions with which they agreed without suggestions for 
changes in those provisions. In most cases, we have not summarized or 
responded to those comments below because they do not require a 
response. However, we appreciate receiving them.

Use of Functional Criteria in the Immune System Disorders Listings

    Comment: Several commenters supported our proposal to add 
functional criteria to each of the listings in this body system. 
However, three other commenters expressed concerns about the proposal. 
One commenter suggested that we should avoid introducing functional 
criteria into

[[Page 14589]]

these listings. The commenter observed that, while the consideration of 
functional impacts may result in greater latitude among adjudicators 
and more flexibility in decisionmaking, there is also an element of 
subjectivity that could result in greater inconsistency in our 
decisions. The second commenter, who generally agreed that 
``functioning should be considered in ratings,'' said that the addition 
of functional criteria to the listings for immune system disorders 
other than HIV infection would not make the evaluation of these 
disorders any easier. This commenter said that considering functional 
information in claimant and third party reports of activities of daily 
living, and treating physician and other source statements would make 
evaluating these disorders more difficult. The commenter also believed 
that more evidence would be needed to support the decisions.
    We address the third commenter's concern in the next comment and 
response.
    Response: As we explained in the NPRM (71 FR at 44440) and earlier 
in this preamble, we are adding the functional criteria in response to 
many comments we received on the ANPRM and in public outreach meetings. 
As many commenters pointed out, the debilitating effects of immune 
system disorders are often ``invisible''; that is, outward signs of the 
disorders and objective severity markers often are not obvious and we 
cannot describe them in a listing. Because of this, the proposal 
received support from many individuals (or their family members) who 
received disability benefits only after going through a long appeals 
process. We also received comments about inconsistencies in our 
adjudications because we did not provide the kinds of guidance about 
evaluating the functional impact of immune system disorders that we do 
in these final rules.
    Therefore, we do not agree with the commenters who thought that 
adding the functional criteria would have the negative effects they 
described or that we should not add functional criteria to these 
listings. To the contrary, we believe that these final listings will 
result in more consistent adjudications, and in some cases, faster 
adjudications, a need for less development, and fewer cases in which 
appeals are necessary, as we explain in more detail below.
    The final listings describe individuals who are very ill. To 
qualify under one of these listings, an individual must first establish 
with objective medical evidence that he or she has the type of immune 
system disorder described by a given listing. Second, the individual 
must show that he or she repeatedly becomes ill as a result of the 
impairment. These two findings alone establish that the individual has 
a significant medical problem. The third requirement, to show a 
``marked'' limitation in at least one of the areas of functioning, 
establishes that the overall impairment causes serious limitations.
    A ``marked'' limitation as we define it is an obvious, serious 
limitation that affects all aspects of the individual's life 
(activities of daily living, social functioning) or the ability to do 
tasks (deficiencies in concentration, persistence, or pace). Therefore, 
it can be easier for an adjudicator to assess whether there is a 
``marked'' limitation in an area of functioning, and to justify that 
assessment, than it is to assess and justify a residual functional 
capacity assessment. Residual functional capacity is more detailed, 
requiring evaluation of specific physical and mental work-related 
functions, what we often call a ``function-by-function'' assessment.
    Because of this, without these final listings, our adjudicators 
would have to do more work in most, if not all, cases of individuals 
who have immune system disorders that will meet these final listings 
only to reach the same decision. Under the prior rules, virtually all 
of the individuals who could now qualify under the new functional 
listings required a residual functional capacity assessment. Our 
adjudicators not only had to do additional work to provide this more 
detailed assessment of functioning, but they also had to do the 
additional work associated with making findings about the ability to do 
past relevant work at step 4 of the sequential evaluation process, and 
to make an adjustment to other work at step 5. Each of these 
determinations--function-by-function residual functional capacity 
assessment, assessment of the ability to do past relevant work, and 
ability to make an adjustment to other work--required development of 
information. We believe that in some cases adjudications under these 
final listings will be easier, faster, and more consistent.
    Finally, we have significant experience applying these and similar 
functional criteria in many claims. We began using these functional 
criteria in listing 14.08 in 1993. We used some of the same criteria to 
evaluate physical impairments in children when we first implemented the 
policy of functional equivalence for children in 1991,\2\ and have used 
similar kinds of criteria for evaluating functional equivalence in 
physical impairment claims since 2000 under Sec.  416.926a of our rules 
(65 FR 54747 (2000)). Many of our listings, including most of our 
musculoskeletal listings, several of our cardiovascular listings, and 
most of the neurological listings, contain functional criteria.
---------------------------------------------------------------------------

    \2\ See generally 56 FR 5534 (1991).
---------------------------------------------------------------------------

    Comment: The third commenter (whose comment was about the 
functional criteria in proposed listing 14.08) suggested that 
limitations in maintaining social functioning and in completing tasks 
in a timely manner due to deficiencies in concentration, persistence, 
or pace are basic issues for evaluating mental impairments under 12.00, 
for mental disorders, and should be removed from the listing. 
Similarly, one of the two commenters whose comments we summarized in 
the preceding comment summary expressed concern that adjudicators could 
assume that the functional criteria in listing 14.08 pertain only to 
the evaluation of mental impairments because they are similar to those 
considered in the context of the mental listings.
    Response: We do not agree that maintaining social functioning or 
completing tasks in a timely manner due to deficiencies in 
concentration, persistence, or pace describe only mental functioning 
and should be removed from listing 14.08K or any of the other 
corresponding final listings. We addressed this issue at length in 1993 
when we first published these rules. In the preamble to the 1993 
publication of the rules, we explained in responding to public 
comments:

    We do not agree that it is inappropriate to apply these 
functional criteria to physical disorders because the criteria are 
generic; they do not describe mental functions, but broad areas of 
functioning that are relevant to any adult's ability to work or any 
child's ability to independently, appropriately and effectively 
engage in age-appropriate activity. * * * [T]hese activities 
describe what people do and how well they do it on a day-to-day 
basis. For our purposes, it is immaterial whether an individual has 
difficulty doing chores or maintaining concentration because of a 
mental disorder or because of fatigue, weakness, pain, headaches, 
frequent diarrhea, or any other physical problem; the person still 
has the limitation that results from a medically determinable 
impairment(s).

58 FR at 36040. We also explained that we had modified the language of 
the introductory text to make it more specific to individuals with HIV 
infection. Those modifications remain in these final rules with even 
further clarifications.
    A number of commenters on the 1993 rules specifically commented 
that the area of social functioning is meant to measure an individual's 
psychiatric

[[Page 14590]]

condition and is not appropriate for the evaluation of HIV. We 
responded that:

    * * * the ability to interact with other people can be affected 
by a physical impairment. For instance, an individual who is 
fatigued may have difficulty going out or sustaining conversation. * 
* *

58 FR at 36041.
    In addition, and as we noted in the response immediately preceding 
this one, over the almost 15 years since we first published listing 
14.08, we have gained considerable experience applying functional 
criteria such as these to physical impairments.
    In final 14.00I, as in the NPRM, we provide that functional 
limitations may result from the impact of the disorder on mental 
functioning, physical functioning, or both mental and physical 
functioning. As we indicated in the NPRM, we revised 14.00I so that it 
applies to all of the listed impairments and more consistently refers 
to symptoms that are related to physical impairments. We believe that 
these revisions will help our adjudicators to better understand and 
remember that the areas of functioning should be applied to physical, 
as well as mental, limitations. However, we will provide training on 
the new functional criteria in these final rules.
    Comment: One commenter said that adjudicators will need guidance on 
how to determine whether to use the immune system disorders listings 
alone versus completing the typical full documentation required for the 
mental disorders listings. The commenter remarked that doing additional 
mental development such as obtaining a consultative examination for a 
mental status examination could potentially delay a claimant's 
determination.
    Response: We agree that guidance is needed and plan to address this 
issue in the training that we will conduct on these final rules. We do 
not believe that mental consultative examinations will be required as a 
result of these final listings because we are not trying to document 
mental impairments. Rather, we are determining any functional 
limitations and restrictions that a person may have as a result of his 
or her immune system disorder(s). As we do for other impairments, such 
as HIV infection, we would expect adjudicators and reviewers to assess 
functioning by evaluating objective medical evidence and evidence from 
other sources as described in Sec. Sec.  404.1512 and 416.912.
    Comment: One commenter suggested that we provide more concrete 
guidance on how to evaluate the severity of limitations in activities 
of daily living and more structure on the application of terms such as 
``moderate, marked, and extreme'' to reduce the likelihood of 
inconsistent interpretation of these terms.
    Response: We did not adopt this comment because the application of 
these terms is often dependent on specific case facts, and because we 
believe that any additional detail would be better presented in 
training and other instructions. Our adjudicators have considerable 
experience evaluating ``marked'' and ``extreme'' limitations and have 
used the functional criteria in prior listing 14.08N which are similar 
to the criteria we include in these final rules. However, we will 
remind adjudicators of our guidance in these areas when we conduct 
training on these final rules.
    Comment: One commenter referred to proposed 14.00I and said that it 
``introduce[d] the concept of `repeated manifestations accompanied by 
functional limitations' '' and the application of this concept to eight 
listings. The commenter observed that this ``new way of evaluating the 
impact of repetitive episodes'' was ``sound in theory'' but ``may be 
difficult to apply in practice'' because of the implicit need to 
document activities of daily living during periods sometimes well in 
the past. The commenter suggested that we clarify that the intent of 
the listings that include standards for evaluating functional 
limitations resulting from repeated manifestations of immune system 
disorders is to document functional limitations occurring in the 
present and does not require extensive documentation of the impact on 
activities of daily living during earlier episodes. The commenter 
indicated that evaluating the impact of repetitive episodes may be 
difficult because of the extended time period for which we may need to 
develop documentation of activities of daily living.
    Response: We believe we accommodated this comment by adding 
language in final 14.00I3 explaining that the manifestation episodes 
must occur within the period covered by the claim. As we already do, 
for example, whenever we need to assess residual functional capacity, 
we will develop evidence about the individual's functioning for the 
entire period covered by the claim. The final rules do not impose any 
additional burden in that regard, as we have explained in our responses 
to the preceding comments.
    Also, we must note that the concept of repeated manifestations 
accompanied by functional limitations is not new. We have used the 
criterion in the HIV infection listings since 1993. The innovation in 
these final rules is to apply the same kind of criterion to the other 
listed immune system disorders.

Systemic Lupus Erythematosus (SLE)

    Comment: One commenter thought that the terms ``repeated,'' 
``marked,'' and ``manifestation'' in the SLE listing could cause 
confusion for physicians and adjudicators. The commenter recommended 
that we clarify the definition of each term or replace the section in 
the SLE listing with a different rule, which the commenter also 
proposed. (We address the proposal to replace the SLE listing in a 
later comment and response.)
    With regard to the term ``marked,'' the commenter believed that our 
proposed definition was ambiguous. The commenter suggested that we add 
more examples of ``marked'' and define it, giving examples of 
``moderate'' for comparison. The commenter also said that physicians do 
not use the term ``marked'' in describing limitations resulting from 
SLE.
    The commenter also suggested that we provide a definition of 
``manifestation'' with examples because it was not defined in the 
proposed rule.
    Response: We do not expect physicians and other medical sources to 
use our terminology. We only need for them to provide us with medical 
evidence that we will use to determine whether an individual's 
impairment meets the requirements of a listing. For example, a 
physician does not need to tell us that a flare of his or her patient's 
SLE was a ``manifestation,'' only report to us what occurred in medical 
terms, and if necessary, provide an opinion that it was related to the 
SLE.
    Likewise, we realize that physicians may not use the term 
``marked'' in describing limitations resulting from SLE. However, for 
the purpose of determining disability, the issue of whether an 
individual has a ``marked'' limitation is an administrative finding 
that we make based upon consideration of all relevant evidence in the 
individual's case record, which may include information that the 
treating source does not have. We only need evidence describing the 
individual's limitations, and we will determine whether those 
limitations meet our definition of ``marked.''
    The definitions of the terms ``repeated'' and ``marked'' in these 
final rules are substantively the same as the definitions of these 
terms in our prior rules, and our adjudicators have been using these 
definitions since 1993, when we issued the prior rules. As we have 
already noted, we use the term ``marked'' in a number of our other 
rules as well.

[[Page 14591]]

    Comment: With regard to the term ``repeated,'' the same commenter 
indicated that patients might not see their physicians often enough to 
satisfy the criterion in the proposed rule, or physicians might not 
record the required information in a patient's chart. The commenter 
said that physicians may not spend time documenting their records 
because of time constraints, and this would be a problem if the 
individual later applies for disability benefits.
    Response: We understand the commenter's concern. However, such 
individuals with SLE can still qualify under final listing 14.02A, 
which does not require a showing of repeated manifestations, and in 
other ways; for example, with impairment manifestations that meet other 
listings, based on our policy of ``medical equivalence,'' or based on 
residual functional capacity. We address the latter issues in final 
14.00G6 for individuals who have not received ongoing treatment or do 
not have an ongoing relationship with the medical community, and final 
14.00J, for individuals whose impairments do not meet the requirements 
of one of these listings.
    Comment: The commenter also said that the requirement for repeated 
manifestations did not recognize that SLE can cause permanent damage 
that remains chronic after the manifestations have stopped. As an 
example, the commenter described an individual who had a severe heart 
attack caused by lupus, who does not experience any new manifestations, 
but who is disabled from permanent heart damage.
    Response: The example of an individual who has permanent, disabling 
heart damage that the commenter provided is an example of the 
principles we discussed in the response immediately above. If the heart 
damage is sufficiently severe, it would meet or medically equal one of 
our cardiac listings in 4.00, the cardiovascular body system. Even if 
it does not meet or medically equal a listing in the cardiovascular 
body system, it could be the basis for a finding of disability at the 
last step of the sequential evaluation process because of the 
functional limitations it causes.
    Also, our criteria for evaluating repeated manifestations of SLE do 
not require repetition of the same manifestation. For example, an 
individual who has experienced three different manifestations of SLE 
(for example, heart problems, leukopenia, and pleuritis) with the 
frequency and duration required in final 14.00I3 would have an 
impairment that satisfies the criterion in final listing 14.02B. In 
response to this comment, we have added language to final 14.00I3 to 
make this clear. This is not a change in what we proposed, only a 
clarification of our intent.
    Comment: The same commenter also suggested that we use the term 
``flare'' instead of ``manifestation'' because that is the word 
physicians treating SLE use to describe increased symptoms and disease 
activity.
    Response: We are aware that physicians who treat SLE often use the 
term ``flare'' to describe increased symptoms and disease activity. 
However, ``flare'' implies a temporary state, and our term 
``manifestation'' does not necessarily mean that. We believe that many 
medical professionals who do not work for us will understand our term, 
but it is not critical that they do.
    Comment: The same commenter provided a suggested replacement for 
the criteria in proposed listing 14.02B that included language such as 
``severe impairment'' in one of the domains and the ``opinion'' of a 
specialist regarding prognosis for improvement in functional capacity. 
The commenter indicated that the proposed criteria were medically 
accurate for evaluating lupus, could be documented through a claimant's 
medical records, and could be easily applied by adjudicators.
    Response: We did not adopt the recommendation for a number of 
reasons. The commenter's criteria included essentially the same 
criteria we had proposed. However, the commenter would have also 
required medical evidence that shows that treatment has not 
significantly reduced the severity of the disorder and is not likely to 
restore the capacity to work. This would have made the listing stricter 
than what we had proposed and stricter than the prior listing.
    Comment: One commenter suggested that we add ``intense generalized 
muscle aches and pains'' to the constitutional symptoms and signs of 
severe fatigue, fever, malaise, or weight loss in proposed listing 
14.02 because it is the most common symptom that rheumatologists who 
treat individuals with lupus hear from their patients.
    Response: We agree that intense generalized muscle aches and pains 
is a common complaint of individuals with SLE. However, these symptoms 
generally respond to treatment. If the muscle aches and pains persist 
or do not respond to treatment, they may be the result of a secondary 
disorder other than SLE. Therefore, we did not adopt this comment.

Systemic Sclerosis (Scleroderma)

    Comment: One commenter suggested that we should make the criterion 
for toe contractures in listing 14.04B1 more specific to make it more 
comparable with the criteria for finger contractures in proposed 
listing 14.04B2, atrophy of the lower extremities in proposed listing 
14.04B3, and atrophy of the upper extremities in proposed listing 
14.04B4. The commenter remarked that ordinary hammer toes are 
contractures and only the most severe result in significant incapacity.
    Response: We did not adopt the comment because we believe that it 
is clear that listing 14.04B1 cannot be met with simple hammer toes. 
The listing requires that the toe contractures be so serious that they 
result in the inability to ambulate effectively. This is consistent 
with listings 14.04B2, 14.04B3, and 14.04B4, which require contractures 
or atrophy with irreversible damage resulting in either the inability 
to ambulate effectively or the inability to perform fine and gross 
motor movements effectively.
    Comment: One commenter pointed out that our inclusion of the phrase 
``or of a toe and finger'' in proposed listing 14.04C1 was redundant 
because we also required that the gangrene must be present in at least 
two extremities. The commenter said that the intent to require two 
extremity involvement is clear and suggested that we remove the rest of 
the language in proposed listing 14.04C1.
    Response: We adopted the comment.

Immune Deficiency Disorders, Excluding HIV Infection

    Comment: One commenter suggested that when we give examples of 
primary immune deficiency disorders in these proposed rules we use 
``Common Variable Immunodeficiency Disorder (CVID)'' instead of the 
word ``agammaglobulinemia'' because it would be less confusing.
    Response: We did not adopt this comment because the example we use 
in these rules is of ``X-linked agammaglobulinemia'' and the term CVID 
does not include this disorder.
    Comment: One commenter suggested that we clarify what constitutes 
``sepsis'' as required in proposed listing 114.07A1 for immune 
deficiency disorders. The commenter remarked that it is not uncommon 
for clinicians to inappropriately label someone as having sepsis or 
urosepsis when the more correct diagnosis was bacteremia with a urinary 
tract infection.
    Response: We did not adopt this comment because we do not agree 
that

[[Page 14592]]

sepsis is commonly misdiagnosed as bacteremia. Additionally, sepsis is 
such a serious condition that we believe that it will be clear from the 
medical records when bacteremia is incorrectly labeled as sepsis.

Human Immunodeficiency Virus (HIV) Infection

General

    Comment: Many commenters suggested that the final rules should 
include enough general language to accommodate the inevitable changes 
in understanding and treatment of HIV infection that will occur during 
the anticipated 8-year life of the rules. The commenters believed that 
we would unfairly deny individuals if we did not include such general 
language and if the individuals' medical records did not include the 
clinical markers required by these listings. The commenters recommended 
that we add a criterion for ``an infection that is systemic or 
disseminated'' to listings 14.08A through F in recognition of these 
anticipated changes. The commenters also suggested that the rules 
should accurately and comprehensively reflect the current understanding 
of HIV disease and treatment.
    Response: The final rules, like the prior rules, do include general 
language that will allow our adjudicators to establish the existence of 
HIV infection and identify manifestations of HIV infection based on 
future advances in medicine and changes in medical science.
     With regard to definitive diagnosis of HIV infection, we 
include in final 14.00F1a(vi) a catchall criterion for ``[o]ther tests 
that are highly specific for detection of HIV and that are consistent 
with the prevailing state of medical knowledge.'' This criterion is 
similar to prior 14.00D3a(iii), and we include it specifically to allow 
for future advances or changes in the methods for diagnosing HIV 
infection.
     Likewise, as in 14.00D3b of the prior rules, we include in 
final 14.00F1b a provision that allows our adjudicators to document HIV 
infection ``without the definitive laboratory evidence described in 
14.00F1a, provided that such documentation is consistent with the 
prevailing state of medical knowledge and clinical practice and is 
consistent with the other evidence in [the individual's] case record.'' 
This permits our adjudicators to establish the existence of HIV 
infection based on current prevailing medical practice and even in the 
absence of laboratory testing. (For an additional explanation of this 
provision when we originally published it in 1993, see 58 FR at 36019 
and 36033.)
     With regard to the manifestations of HIV infection, the 
language in these final rules is general. For example, final 14.00F3a 
requires only definitive documentation ``by culture, serologic test, or 
microscopic examination of biopsied tissue or other material.'' Final 
14.00F3b contains virtually the same language as in final 14.00F1b 
regarding other acceptable documentation of the manifestations of HIV 
infection.
    Additionally, we did not add the recommended listing criterion for 
two reasons. First, the listings are only examples of impairments that 
we consider severe enough to prevent any gainful activity and are not 
meant to be an all-inclusive list of such impairments. If an individual 
with HIV infection has an opportunistic disease or other condition that 
is not listed, we will consider whether it medically equals any 
listing; that is, whether it is as medically severe as an impairment in 
the listings. Second, if we added the language proposed by the 
commenters we might inadvertently include some persons who do not have 
listing-level impairments.
    It is also important to remember that we do not deny benefits to 
anyone simply because his or her impairment(s) does not meet or 
medically equal the severity of a listing. We may still find such an 
individual disabled based on other rules in the appropriate sequential 
evaluation process for adults or children.
    We do, however, agree that the listings should reflect the latest 
medical knowledge of HIV infection. As noted earlier, we are publishing 
separately an ANPRM in today's edition of the Federal Register inviting 
comments and suggestions on how to update and revise our listings for 
HIV infection. We believe that we need additional information before 
considering whether to propose additional changes to the criteria in 
the HIV infection listings.
    Comment: Many commenters suggested that we add guidance to 
acknowledge that disability may result from conditions that are not 
specified in these final listings or that may emerge as a result of new 
or sustained HIV treatment by adding the following guidance: ``Special 
consideration should be given to other conditions, signs and symptoms 
deemed by the primary care provider as contributing to substantial 
functional limitations.''
    Response: We did not adopt these comments. The final listing--like 
the prior listings--already allows for the consideration of conditions 
that are not specified and that may arise in the future. The opening 
paragraph of final 14.08K explains that HIV manifestations considered 
under this listing can be the manifestations listed in 14.08A-J ``or 
other manifestations,'' and then provides a parenthetical list of 
examples of such other manifestations. Since the parenthetical list 
says ``for example,'' the listing does include any other manifestations 
of HIV infection, including new manifestations that may arise in the 
future. The nature of the manifestation is less important than the fact 
that the individual repeatedly experiences them.
    We did not include the phrase ``deemed by the primary care provider 
as contributing to substantial functional limitations'' because the 
statement is not an accurate characterization of how we determine the 
existence and severity of impairments, impairment manifestations, and 
functional limitations, or of how we consider medical opinions from 
treating sources. We have other, general rules that explain these 
policies, and it would not be appropriate to repeat them in a listing.
    Also, if a new manifestation should arise in the coming years, we 
will still be able to tell our adjudicators about it through internal 
guidelines we can issue. We can also provide training if necessary.
    Comment: Many commenters suggested that these rules should address 
the interplay between HIV and mental health. The commenters said that 
the rules should recognize that mental health conditions can be a 
manifestation of HIV infection which, even if they do not meet or 
medically equal mental disorders listings, should be considered as 
repeated manifestations of HIV infection. They also said that the rules 
should indicate that attention must be paid to the signs and 
limitations that stem from mental and emotional deficits when 
evaluating the severity and level of progression of HIV disease.
    Many commenters remarked that HIV medications can themselves cause 
mental impairments, such as significant memory loss, cognitive 
deficits, depression, anxiety, paranoia, and hypervigilance. These 
commenters also indicated that mental illness may become more 
pronounced as the HIV disease progresses and can interfere with self-
care, activities of daily living, and adherence to treatment regimens 
and appointment schedules. The commenters suggested that primary care 
providers and infectious disease specialists may prescribe compensatory 
medications, such as anti-depressants and anti-anxiety medication, to 
their

[[Page 14593]]

patients without referring them for psychiatric care or counseling. 
They said that, in such cases, there will be no longitudinal history of 
psychiatric care or assessment, but that we should recognize these 
manifestations of HIV infection which contribute to the disabling 
nature of the disease. The commenters suggested that we add another 
subsection to final 14.00F to make these points and that we revise 
listings 14.08K and 114.08L to recognize specifically that mental 
health conditions can be a manifestation of HIV infection that can be 
considered under those listings.
    Response: We did not agree with these comments, but we clarified a 
phrase in the final rules in response to them. The proposed rules did, 
and these final rules do, recognize the interplay between HIV infection 
and mental health, and that mental health conditions can be 
manifestations of HIV infection. While we did indicate in proposed 
14.00J2 that individuals with immune system disorders ``including HIV 
infection'' may manifest signs or symptoms of a mental impairment that 
could be evaluated under the mental disorders listings, we also made 
provision throughout the immune system disorders listings for 
individuals whose mental impairments would not meet or medically equal 
a mental disorders listing, and recognized that mental limitations 
could result from HIV infection or its treatment.
    First and foremost, we included ``cognitive or other mental 
impairment'' as an example of a manifestation of HIV infection that 
would satisfy the requirement for repeated manifestations in proposed 
listing 14.08K. We also provided in proposed 14.00G1, 14.00G5, and 
their corresponding childhood sections that limitations in mental 
functioning can be a side effect of treatment for immune system 
disorders, while in proposed 14.00I4 and 114.00I3 we indicated that 
mental limitations can result from the impact of the disease process 
itself. All of these provisions are in the final rules.
    We did not add some of the other information the commenters 
suggested because we believe that it is too detailed for inclusion in 
our listings, and some of the proposals also would apply to our 
evaluation of other immune system disorders as well as HIV infection. 
However, we will consider including this guidance in the training we 
provide for our adjudicators on these listings.
    However, in response to these comments, we changed the phrase 
``cognitive or other mental impairment'' in proposed 14.08K to 
``cognitive or other mental limitation'' in final 14.08K. This should 
help to clarify that we will consider cognitive or other mental 
limitations as manifestations under this listing regardless of whether 
the existence of a ``mental impairment'' (that is, a mental condition) 
has been established.
    Comment: Many commenters suggested that we make it clear throughout 
the proposed rules that each claimant is entitled to an individualized 
assessment of his or her HIV infection.
    Response: We did not make any changes in response to this comment. 
The commenters did not provide examples of sections of the rules that 
they thought should be improved and did not recommend specific 
revisions, and we believe these final rules do make clear that we 
require an individualized assessment of an individual's HIV infection 
or any other immune system disorder. For example, the rules stress the 
importance of considering the individual's symptoms and limitations 
caused by the disease or its treatment. Also, individualized assessment 
is a general principle that applies throughout all of our disability 
rules.
    Comment: Two commenters questioned our decision to not make any 
substantive changes to the proposed HIV infection listings that require 
HIV infection and certain opportunistic infections, such as the listing 
for PCP. The commenters indicated that there have been advances in the 
understanding and treatment of HIV infections since these listings were 
originally published. One commenter remarked that the widespread 
availability of highly active antiretroviral therapy (HAART) has 
changed the occurrence and progression of complications of HIV 
infection and that scientific advances have permitted the dosing of 
much fewer pills than previously required. Other commenters, including 
a medical association representing HIV medical providers, supported our 
decision not to change the stand-alone listings contained in listing 
14.08.
    Response: As noted in the NPRM, we carefully considered the 
advances in treatment and consequent increases in longevity that have 
occurred since we published the prior rules in 1993. Based on this 
review, we did not believe that there had been sufficient progress in 
the treatment and control of HIV infection to warrant any change in 
these rules at that time. However, as a result of public comments on 
the NPRM, we now believe that some changes may be appropriate. 
Therefore, as noted above, we are publishing separately an ANPRM in 
today's edition of the Federal Register inviting comments and 
suggestions on how we might update and revise our listings for HIV 
infection. We will consider the comments and suggestions that we 
receive in response to the ANPRM, as well as our adjudicative 
experience and additional information about advances in medical 
knowledge, treatment, and methods of evaluating HIV infection. If we 
determine that listing 14.08 should be further revised, we will publish 
for public comment an NPRM that will propose specific revisions to the 
listing.
    Comment: Three commenters suggested that there should be a time 
period for reviewing claims allowed under proposed listing 14.08, such 
as a period of 12 months or 3 years, similar to the time period we have 
in some other listings, such as organ transplants and malignant 
neoplastic diseases.
    Response: We did not adopt this comment. The disease process for 
HIV infection is not the same as it is for disorders such as organ 
transplants or malignant neoplastic diseases, and we do not believe the 
use of timeframes for the HIV infection listings would be appropriate 
at this time.

Manifestations of HIV Infection

    Comment: One commenter suggested, without explanation, that we 
modify the criteria in proposed listing 14.08A1 by eliminating the 
requirement that pulmonary tuberculosis be ``resistant to treatment.''
    Response: We did not adopt this comment. We added pulmonary 
tuberculosis resistant to treatment in 1993 in response to public 
comments. (58 FR at 36021) We are unaware of changes in medical science 
or treatment since then that would indicate that we should consider 
pulmonary tuberculosis that is responsive to treatment to be of 
listing-level severity, and the commenter did not provide a reason for 
the recommendation.
    Comment: One commenter suggested that we include esophageal 
candidiasis in the examples of those conditions in final 14.00F3b for 
which a presumptive diagnosis can be made. The commenter indicated 
that, like PCP, CMV diseases, and toxoplasmosis of the brain, 
esophageal candidiasis is typically diagnosed based on clinical 
manifestations, history, and treatment response, and that when it is, 
it will meet listing 14.08B2. Another commenter made a similar comment 
and suggested that we include information about medical and other 
evidence that could be used to presumptively diagnose Candida 
esophagitis, similar to the guidance in 14.00F3b(i) for PCP. This 
commenter suggested that such guidance would

[[Page 14594]]

remind our adjudicators that a diagnosis of ``Candida esophagitis'' 
without supporting medical evidence is insufficient to meet or 
medically equal listing 14.08B2.
    Response: We adopted these comments by adding new paragraphs 
14.00F3b(iv) and 114.00F3b(iv). They describe other acceptable evidence 
that we may use to document the presence of candidiasis of the 
esophagus, also known as Candida esophagitis. We agree with the first 
commenter that presumptively diagnosed Candida of the esophagus meets 
the requirements of the listing. We also agree with the second 
commenter that a diagnosis alone is not sufficient to establish 
disability under the listing; we must have medical evidence to support 
the diagnosis. We did not state this in the new paragraph because it is 
a basic principle in our disability programs, applicable to any 
impairment.
    In the new paragraphs, we provide guidance indicating that typical 
treatment response ``can be supportive of the diagnosis,'' consistent 
with the first commenter's recommendation. For consistency, we added 
the same guidance in final 14.00F3b(i) and 114.00F3b(i) in the 
statement about treatment response for PCP.
    Comment: One commenter suggested that the guidance in proposed 
14.00F3b(i) for documenting the diagnosis of PCP without definitive 
laboratory evidence was questionable and insufficient. The commenter 
remarked that the diagnosis of PCP should be documented on the basis of 
prevailing and accepted medical knowledge, and that the discussion in 
this proposed section should otherwise be deleted.
    Response: We did not agree with this comment. The criteria we 
included in the NPRM and these final rules are appropriate examples of 
medically accepted supportive evidence of PCP infection.\3\ However, in 
response to this comment we are adding ``no evidence of bacterial 
pneumonia'' in final 14.00F3b(i) and 114.00F3b(i) as another piece of 
supportive evidence that may be used to diagnose PCP presumptively.
---------------------------------------------------------------------------

    \3\ See Cecil Textbook of Medicine at 2059-2064 (Lee Goldman and 
Dennis Ausiello, eds.22nd ed., 2004).
---------------------------------------------------------------------------

    Comment: One commenter suggested that we change the reference to 
``Pneumocystis carinii pneumonia (PCP)'' in proposed 14.00F1b to 
``PneumoCystis Pneumonia (PCP) caused by infection with Pneumocystis 
jiroveci'' to be more consistent with prevailing medical knowledge. The 
commenter also suggested that we change the criteria of ``Pneumocystis 
carinii (jiroveci) pneumonia or extrapulmonary pneumocystis carinii 
(jiroveci) infection'' in proposed listing 14.08B7 to ``PneumoCystis 
Pneumonia (PCP) or extrapulmonary pneumocystis infection caused by 
Pneumocystis jiroveci.''
    Response: We partially adopted the comment. In final 14.00F1b and 
final listing 14.08B7, we now refer to ``Pneumocystis pneumonia (PCP)'' 
to reflect current medical terminology. Because of this change, we also 
removed the note we had proposed to include in 14.00F3b(i) which 
explained that ``Pneumocystis carinii'' is now known as ``Pneumocystis 
jiroveci'' and that ``PCP'' remains in common usage for the pneumonia 
caused by this organism. We no longer need the note because we no 
longer refer to Pneumocystis carinii or Pneumocystis jiroveci in these 
rules. We also made corresponding changes in the childhood introductory 
text.
    Comment: One commenter suggested that we include an authoritative 
source for moving prior listing 14.08B7 for PCP from the section of the 
listings for protozoan and helminthic infections to the section of the 
listings for fungal infections.
    Response: When we published the NPRM, we listed the references that 
we consulted when we were developing the proposed rules (71 FR at 
44448). This list included ``Medical Management of HIV Infection'' 
(Johns Hopkins University 2003) by J.G. Bartlett and J.E. Gallant, 
which classifies Pneumocystis carinii as a fungal infection.
    Comment: One commenter suggested that we modify the language in the 
next to the last sentence in proposed 14.00F3b(ii) to clarify that we 
do not require the presence of all of the signs noted in this sentence 
to support a presumptive diagnosis of Cytomegalovirus by indicating 
that the supporting evidence ``may'' include the findings we listed.
    Response: We adopted the comment. As we noted in the summary of the 
final rules earlier in this preamble, we are also adding the word 
``may'' in final 14.00F3b(i), for PCP, to be consistent with this 
change.
    Comment: One commenter suggested that we clarify whether the intent 
of proposed listing 14.08E4, for squamous cell carcinoma of the anus, 
was to include both anal canal cancers and anal margin tumors or to 
limit the listing solely to anal canal cancers (developing from 
mucosa).
    Response: We adopted the comment by changing the criterion to 
``Squamous cell carcinoma of the anal canal or anal margin'' in final 
14.08E4 and 114.08E4. This is not a substantive change, but only 
clarifies our intent.
    Comment: Many commenters said that we should revise the criteria in 
proposed listing 14.08H for evaluating HIV wasting syndrome to reflect 
more current medical knowledge about this condition. They said that we 
should provide that body mass index (BMI) and body cell mass (BCM) can 
be relied upon as accurate indicators of the severity of wasting in a 
given individual. They also said that this listing is too restrictive 
in its documentation requirements, and that involuntary weight loss as 
low as 5 percent has been associated with increased risk of death. 
Another commenter suggested that we revise the criteria for this 
listing to ``HIV wasting syndrome, characterized by involuntary weight 
loss of 5 percent or more below ideal body weight within six months 
and, in the absence of concurrent illness that could explain the 
findings.'' The commenter said that this would reflect medical 
guidelines for diagnosing the condition and the significance of rapid, 
unintentional weight loss.
    Most of the commenters also said that the prior requirements for 
diarrhea were too restrictive because a person with HIV infection who 
experiences wasting is functionally unable to work if he or she 
experiences diarrhea for 2 weeks and protein deficiency. They also said 
that, although a documented fever is a useful clinical indicator of 
wasting syndrome, the listing should not require the individual to have 
``many temperature readings throughout a month or for a longer 
period.'' They said that HIV wasting syndrome can be disabling even in 
the absence of the listing requirement when it is accompanied by 
constitutional symptoms, such as weakness, lack of muscle strength, 
fatigue, malaise, or inability to lift. They suggested that as an 
alternative to evidence of diarrhea or fever, the listing could contain 
language comparable to that in proposed 14.00F; that is, ``documented 
by other generally acceptable methods consistent with the prevailing 
state of medical knowledge or clinical practice.''
    Response: We agreed with the commenters who suggested that we 
include a reference to BMI in the listing, and have clarified final 
listing 14.08H by explaining that we can compute the 10 percent loss of 
weight in pounds, kilograms, or by BMI. We did not add a reference to 
BCM because BCM is more of a research concept, involves calculations of 
body composition, and is not in wide usage in the general medical 
community.

[[Page 14595]]

    We also added guidance in final 14.00F5 to remind adjudicators that 
they can evaluate HIV infection that affects the digestive system and 
results in malnutrition under listing 5.08. Even though there is no 
listing for ``wasting syndrome'' in part B, there is a criterion in 
final listing 114.08H3, the growth disturbance listing, for a loss of 
10 percent of body weight. We have added the same statement about 
pounds, kilograms, and BMI in that final rule as well, and a statement 
referring to listing 105.08 in the digestive system at the end of final 
114.00F4a.
    We did not make other changes in these final listings in response 
to the comments. We use listings to find individuals whose impairments 
are so severe that we do not need to consider their age, education, and 
previous work experience to decide that they are disabled. We believe 
that, while some individuals with the findings recommended by the 
commenters will be disabled under our rules, and some will be at risk 
of dying, others will not, so we cannot presume disability based on 
those findings in all, or even most, individuals. Even if they are 
initially unable to work, we believe that many individuals with the 
findings suggested by the commenters will not have impairments that 
meet the duration requirement in the Act and our regulations, that is, 
have an impairment that is expected to result in death or that has 
lasted or can be expected to last for a continuous period of not less 
than 12 months.
    However, some individuals with a 5 percent weight loss will have 
impairments that meet the requirements of listing 14.08H; in some 
individuals, a 5 percent weight loss will be a ``significant 
involuntary weight loss.'' As we explain in final 14.00F5, final 
listing 14.08H does not require a specific minimum amount or percentage 
of weight loss. We always consider an involuntary weight loss of at 
least 10 percent of baseline ``significant,'' but an involuntary weight 
loss of less than 10 percent may also be ``significant'' depending on 
the individual's baseline weight and body habitus. We also provide 
examples in final 14.00F5 of when weight loss of less than 10 percent 
of body weight may and may not be significant.
    Likewise, although we agree that an individual with HIV infection 
who experiences diarrhea for 2 weeks with protein deficiency would have 
work-related limitations, and may be unable to work for a time, we do 
not believe that this finding by itself would necessarily be indicative 
of an impairment that would be expected to result in death or prevent 
the ability to work for a continuous period of at least 12 months. We 
must consider the specific facts of such individuals' cases to decide 
whether they are disabled.
    With regard to the comment about fever, we did not include a 
requirement in the prior rule or proposed rule, nor do we include one 
in the final rule, for the number of times during the course of a month 
in which the individual's temperature must be taken. We must only have 
sufficient information to determine that the individual has had a 
persistent fever throughout most of a month. More importantly, the 
criterion for fever in final listing 14.08H2 is only one of two 
criteria in listing 14.08H by which an individual may qualify, so an 
individual could qualify under this listing without fever. We believe 
that the fever criterion is medically supportable as an indicator of an 
HIV infection of listing-level severity when considered in the context 
of the other criteria of involuntary weight loss and chronic weakness. 
Also, an individual with wasting syndrome could qualify without a 
finding of fever and with the kinds of constitutional symptoms and 
signs suggested by the commenters under final listing 14.08K.
    We also did not add language that is comparable to that in proposed 
14.00F as an alternative to the evidence of diarrhea or fever because 
the criteria in final listings 14.08H1 and 14.08H2 are severity 
criteria. The language proposed by the commenters would only help to 
establish the diagnosis of wasting syndrome and would not be sufficient 
to establish severity or duration under the listings.
    However, as we noted earlier, we are publishing separately an ANPRM 
in today's edition of the Federal Register inviting comments and 
suggestions on how we might update and revise our listings for HIV 
infection. We believe that we need additional information before 
determining whether to propose any substantive changes to the criteria 
in the HIV infection listings.
    Comment: Many commenters said that we should modify proposed 
listing 14.08I to reflect current medical views regarding diarrhea and 
its treatment. They said that many patients with disabling diarrhea do 
not require hydration and therefore are not treated with intravenous 
hydration, and that ``tube feeding'' is rarely used now to treat 
diarrhea.
    The commenters said that diarrhea can rise to the level of being 
disabling without the objective findings in proposed listing 14.08I. 
They suggested that this listing should include individuals who have 
multiple loose stools each day, bowel incontinence, or a combination of 
the two, despite modifications in HAART and antidiarrheals. They also 
suggested that we should allow documentation by other objective 
evidence, such as reports of a rectal examination, stool culture, or 
fecal occult blood test. Finally, they recommended that we add language 
comparable to that in proposed 14.00F; that is, ``documented by other 
generally acceptable methods consistent with the prevailing state of 
medical knowledge or clinical practice.''
    Response: We did not adopt the comments in these final rules. While 
we agree that many individuals with chronic diarrhea do not need 
hydration and that tube feeding is rare, these criteria provide some 
objective verification of the chronicity and severity of the diarrhea 
and our adjudicative experience shows that individuals do qualify based 
on the criteria. We did not adopt the criteria the commenters proposed 
because we believe that they are not sufficient to reliably document 
the severity, frequency, and chronicity of the diarrhea for our 
disability evaluation purposes. We also believe that the other 
objective evidence the commenters proposed (that is, rectal 
examination, stool culture, and fecal occult blood testing) would not 
be sufficient for this purpose. Lastly, we did not adopt the comment 
asking us to add language to proposed 14.00F because it would only help 
to establish the existence of the impairment, not its frequency and 
chronicity.
    Comment: One commenter suggested that we should characterize the 
symptom of ``fatigue'' in listing 14.08K as ``severe fatigue'' to 
reflect a symptom at listing-level severity and to be consistent with 
the other immune system disorders listings.
    Response: We adopted the comment. The change is not substantive, 
but only a clarification. Like the prior rule and the proposed rule, 
the final rule specifies that the symptoms listed must be 
``significant.'' Therefore, adding ``severe'' does not change its 
meaning. For consistency, we added the word ``severe'' before the word 
``fatigue'' throughout these final rules.
    Comment: One commenter asked why we limited proposed listing 
114.08A4 to children less than 13 years of age, particularly when 
proposed 114.00F4c said that children age 13 and older may have an 
impairment that medically equals this listing. The commenter noted that 
there is nothing in the listing to alert one to the possibility of a 
medical equals for older children.

[[Page 14596]]

    Response: We partially adopted the comment. The age 13 cutoff has 
been in this listing since we first published it in 1993. When we first 
published it, we explained in the preamble to the regulations that 
these types of infections are more serious and more indicative of a 
rapid decline in younger children, that we had considered a younger age 
cutoff, but that we decided on age 13 as a medically appropriate 
dividing line. See 58 FR at 36047.
    The impact of pyogenic bacterial infections in children who are 
under the age of 13 is usually more harmful than in older children, and 
there is general medical acceptance for evaluating the severity of 
these infections differently depending on the age of the child. 
Therefore, we did not change the age requirement in this listing. 
However, in response to this comment, we added a reference to 114.00F4c 
in final listing 114.08A4 to remind adjudicators that children age 13 
and older may medically equal this listing.

Suggested Additional Criteria for the Listing for HIV Infection

    Comment: One commenter suggested that we ``acknowledge'' in final 
14.00F2 that a CD4 count of 100 or less would document the severity or 
functional limitations of HIV infection and establish disability. The 
commenter remarked that the CDC classifies a person with HIV and a T-
cell (CD4) count below 200 as having AIDS and that the susceptibility 
to illness for such individuals increases dramatically. The commenter 
also indicated that a person with HIV and a CD4 count below 100 is 
likely to exhibit an extreme susceptibility to opportunistic infections 
and disabling illnesses, have difficulty tolerating medication, 
experience graver physical conditions, and exhibit lower functional 
capacities than individuals with stronger immune responses.
    Response: We did not adopt this comment. We agree that a CD4 count 
of 100 or less indicates an increased susceptibility to developing 
opportunistic infections and is an important finding when considering 
treatment options. However, we do not agree that CD4 counts are a good 
indicator of disability. We continue to have the same opinion we had 
when we published the prior rules in 1993. In the preamble to those 
rules, we explained that:

while a low CD4 count (and especially a rapidly declining CD4 count) 
is an indicator of a compromised immune system and a valuable tool 
for determining when to institute prophylactic treatment, there is 
no consistent correlation between a given CD4 count and how or 
whether an individual is functionally impaired by HIV infection. 
Individuals with high CD4 counts may be quite severely limited, 
while others with very low counts may be able to continue normal 
activities. One individual who commented on our proposed rules 
related his own story of living with HIV infection, noting that he 
continued to feel well and to work until his CD4 count was well 
below 100. He argued that to base our rules on such an unreliable 
indicator would be to unfairly stigmatize individuals who are able 
to function well despite low CD4 counts.

58 FR at 36018.\4\
---------------------------------------------------------------------------

    \4\ See also 58 FR at 36038, where we provided the same 
information in our response to the public comments about this issue.
---------------------------------------------------------------------------

    There have been significant advances in treatment and monitoring of 
individuals with HIV infection since we published the prior rules in 
1993. Therefore, we believe that what we said in 1993 is, if anything, 
even more relevant to our disability adjudications today.
    Comment: One commenter suggested, without explanation, that we add 
``Rhodococcus'' to the criteria of listing 14.08A for bacterial 
infections, ``Blastomycosis'' and ``Penicillium marneffei'' to the 
criteria of listing 14.08B for fungal infections, and ``Leishmaniasis'' 
and ``Microsporidiosis'' to listing 14.08C (protozoan or helminthic 
infections).
    Response: We did not adopt these comments. We did include 
``microsporidiosis'' in proposed, now final, listings 14.08C1 and 
114.08C1; it was also in prior listings 14.08C1 and 114.08C1. We did 
not add the other suggested manifestations because the listings are 
only examples of impairments that we consider severe enough to prevent 
any gainful activity and are not meant to be all-inclusive. Also, if an 
individual with HIV infection has an opportunistic disease or other 
condition that is not listed, we will consider whether it medically 
equals a listing.
    Comment: Many commenters suggested that the criteria in proposed 
listing 14.08D, for viral infections, should include individuals who 
have both HIV infection and hepatitis B or hepatitis C under listing 
14.08D. The commenters said that individuals who are infected with both 
HIV and hepatitis are more prone to illness, more difficult to treat, 
and less able to function than individuals who are only infected with a 
hepatitis virus. They also indicated that co-infection with HIV and 
hepatitis B or C complicates the treatment of both conditions.
    Response: We did not adopt this comment. While we agree that co-
infection with HIV infection and hepatitis B or C may complicate the 
treatment of these conditions, increase susceptibility to illness, and 
impact functioning, we also believe that the severity of the co-
infection will vary from individual to individual and may not result in 
disability. Because of this, we believe that each claim involving this 
co-infection must be evaluated on a case-by-case basis. This includes 
evaluating whether the co-infection results in manifestations that 
would satisfy the criteria in final listings 14.08K or 114.08L.
    However, we do provide in final 14.00G1f and 114.00G1f that the 
interactive and cumulative effects of treatments for co-occurring 
impairments, such as treatment for HIV infection and hepatitis C, may 
be greater than the effects of each treatment considered separately.
    Comment: Many commenters said that we should add a stand-alone 
listing for chronic or severe acute pancreatitis under proposed listing 
14.08. The commenters indicated that pancreatitis is frequently 
associated with HIV infection, can be caused by HIV infection or 
medications used to treat HIV infection, and may severely impair an 
individual's ability to function. They also said that pancreatitis can 
cause severe and recurring manifestations, such as abdominal pain, 
nausea, vomiting, fever, chills, and shortness of breath, that can 
result in a hospital admission for 2 or 3 weeks at a time or in 
profound weight loss and long-term food intolerance.
    One commenter suggested that we specify under this listing that an 
individual with HIV infection is disabled if he or she requires 
hospitalization for pancreatitis twice in a 1-year period. Other 
commenters suggested that we include a listing that is satisfied by 
evidence of one or more episodes of pancreatitis from which clinical 
recovery is incomplete after 6 months and is accompanied with disabling 
symptoms such as, but not limited to, abdominal pain, diarrhea, 
significant weight loss, nausea, anorexia, and glucose intolerance 
requiring frequent monitoring or treatment.
    Response: We did not adopt the comments. Generally, pancreatitis in 
individuals with HIV infection is caused by HAART and is acute; the 
pancreatitis usually resolves after HAART is suspended briefly. Because 
of this, it would not be appropriate to add a stand-alone listing for 
episodes of pancreatitis or the other criteria recommended by the 
commenters. The criteria recommended by the

[[Page 14597]]

commenters would not necessarily result in the inability to do any 
gainful activity for a continuous period of at least 12 months as 
required by the Act.
    However, individuals with pancreatitis can qualify under these 
listings. As we did in the NPRM, we include pancreatitis as an example 
of an ``other manifestation'' under final listing 14.08K. (We do not 
refer to it in 114.08L because pancreatitis is not as frequent a 
problem in children as it is in adults. However, since the list of 
other manifestations is only a list of examples, pancreatitis is still 
included.) Many individuals who experience pancreatitis with the 
significant accompanying problems described by the commenters will also 
have serious functional limitations and will be able to qualify under 
final listing 14.08K. Individuals with problems such as profound weight 
loss with prolonged food intolerance may have impairments that meet or 
medically equal the requirements of other HIV infection listings or 
listings in other body systems; for example, listings 5.08 and 105.08 
for weight loss. We may also find that they qualify based on an 
individualized assessment of residual functional capacity if there is 
an inability to work or, for children, functional equivalence.

Effects of Treatment for HIV Infection

    Comment: Many commenters suggested that in proposed 14.00G5 and 
114.00G5 we should directly address the issue of a claimant's non-
responsiveness to HIV treatments and specifically state that the mere 
fact that an individual fails to respond to HAART does not indicate 
that he or she is not disabled or is not credible. They also suggested 
that we add a subsection addressing the fragility of persons who do not 
respond to prescribed treatment and the impact of reduced treatment 
options on them. The commenters noted that we addressed these issues in 
the ``general section'' on response to treatment (that is, 14.00G2 and 
114.00G2) but thought that we should address these issues specifically 
for HIV infection in 14.00G5 and 114.00G5.
    Response: We did not adopt these comments. As the commenters noted, 
we provide guidance in 14.00G2 and 114.00G2 that response to treatment 
and adverse or beneficial consequences of treatment may vary widely. 
These sections explain that we consider a variety of factors when 
evaluating response to treatment, including the limited number of drug 
combinations that may be available for treatment, and that we must 
consider the effects of treatment on an individual basis. We also 
provide a specific example of an individual with HIV infection whose 
impairment does not respond to antibiotics or who develops a resistance 
to treatment that had worked in the past.
    We included this new guidance in our rules to address the major 
issues that are raised in these comments, and we believe that it will 
help to respond to the concerns that the commenters raised, not only 
for individuals who have HIV infection but for individuals with other 
kinds of immune system disorders who experience the same kinds of 
problems. Therefore, we do not believe that there is a need to repeat 
this guidance specifically for HIV infection in final 14.00G5 and 
114.00G5 at this time.
    Comment: Many commenters suggested that we revise proposed 14.00G5 
to address the difficulty of adhering to HIV treatment regimens, and to 
acknowledge that there are many valid reasons why individuals with HIV 
infection do not strictly adhere to their prescribed treatment 
regimens. They also suggested that the rules state that a claimant's 
admitted lack of adherence to HAART should neither reflect on the 
claimant's credibility nor indicate that his or her functional capacity 
is ``artificially low.'' They indicated that claimants should not be 
penalized for their failure to adhere to complicated medication 
regimens.
    Response: We partially adopted the comment. We agree that some 
individuals may have difficulty adhering to their treatment regimens 
for HIV infection, such as HAART, and that there may be valid reasons 
for their lack of adherence, such as side effects of treatment (for 
example, diarrhea, nausea, vomiting, neuropathy, or severe fatigue). We 
addressed this in proposed, now final, 14.00G to an extent, especially 
in 14.00G1 and 14.00G2, in which we provided a list of things that we 
consider when we evaluate the effects of treatment. We also have other 
rules that tell our adjudicators not to make the kinds of presumptions 
that concerned the commenters. For example, our regulations on 
evaluating residual functional capacity, Sec. Sec.  404.1545 and 
416.945, provide that adjudicators must consider all relevant evidence 
in determining a person's functional abilities; this means that they 
cannot draw conclusions only from the fact that an individual is not 
receiving or following treatment. In Social Security Ruling (SSR) 96-
7p, we provide that, when we consider treatment in assessing an 
individual's statements about symptoms, ``adjudicator[s] must not draw 
any inferences about an individual's symptoms and their functional 
effects from a failure to seek or pursue regular medical treatment 
without first considering any explanations that the individual may 
provide, or other information in the case record, that may explain 
infrequent or irregular medical visits or failure to seek medical 
treatment.'' One of the examples of a good explanation that we provide 
in the SSR is ``[t]he individual may not take prescription medication 
because the side effects are less tolerable than the symptoms.'' \5\
---------------------------------------------------------------------------

    \5\ See ``Titles II and XVI: Evaluation of Symptoms in 
Disability Claims: Assessing the Credibility of an Individual's 
Statements,'' 61 FR 34483 (1996). Also available at: http://www.socialsecurity.gov/OP_Home/rulings/di/01/SSR96-07-di-01.html.
---------------------------------------------------------------------------

    However, in response to this comment, we added a sentence to final 
14.00H and 114.00H that is based on the sentence from SSR 96-7p quoted 
above. We chose this section for the new sentence because we believe 
that the issue that concerned the commenters will arise most often when 
we are evaluating symptoms and their functional effects. We did not add 
the more detailed information the commenters asked us to include 
because we determined that it would be too extensive to include in the 
final listing. However, we will address the issue in training and 
consider whether to provide written guidance in our internal 
instructions as well.
    Comment: One commenter suggested that we expand proposed 14.00G5a 
to discuss the disfiguring aspects of treatment as an adverse effect of 
treatment. The commenter remarked that adverse reactions to treatment, 
such as ``buffalo hump'' and other fat redistribution can have a 
significant impact on the ability of a claimant who is HIV positive to 
function physically, as well as on his or her emotional well-being.
    Response: We partially adopted this comment. We added a 
parenthetical statement in final 14.00G5a and 114.00G5a to clarify that 
``lipodystrophy'' means fat redistribution. We also cite ``buffalo 
hump'' as an example of fat redistribution.
    In addressing this comment, we also noticed that in the last 
sentence of the paragraph, where we referred to limitations from HIV 
infection, we mentioned only limitations that result from symptoms. 
Since the objective effects of HIV infection can also cause 
limitations, we expanded this sentence to include ``signs'' of HIV 
infection. We do not believe other changes are needed because the 
sentence also refers to the

[[Page 14598]]

side effects of treatment, which includes ``buffalo hump.''

Inflammatory Arthritis

    Comment: One commenter recognized that we had removed reference 
listings and that we provided guidance for using appropriate listings 
in the introductory text. Nevertheless, the commenter suggested that in 
listing 14.09 we refer adjudicators to listings 1.02 and 1.03 when 
involvement of only one major lower extremity joint results in 
ineffective ambulation.
    Response: We adopted the comment by revising the listing so that it 
is no longer necessary for adjudicators to refer to listing 1.02 or 
1.03. As a consequence of this change, we also removed proposed 
14.00D6e(iv) and 14.00D6e(v).
    The commenter was referring to an anomaly in our prior rules. Like 
the prior listing, proposed listing 14.09A required inflammatory 
arthritis with involvement of two or more peripheral weight-bearing 
joints that resulted in an inability to ambulate effectively. However, 
some individuals who have involvement of only one major peripheral 
weight-bearing joint have an inability to ambulate effectively. Under 
the proposed listing and our prior rules, these individuals qualified 
under listing 1.02 in the musculoskeletal system, which specifies that 
the listing is met with ``involvement of one major peripheral weight-
bearing joint.'' In reviewing this comment, we determined that it would 
be simpler if we included a provision similar to that in listing 1.02 
under listing 14.09A. This inclusion allows our adjudicators to use the 
inflammatory arthritis listing for all individuals who have 
inflammatory arthritis that results in an inability to ambulate 
effectively.
    Likewise, the proposed rules and our prior rules made a distinction 
between individuals with inflammatory arthritis who had persistent 
deformity without ongoing inflammation (evaluated under listing 1.02) 
and those who had ongoing inflammation (evaluated under prior listing 
14.09). In reviewing the proposed rules in light of the comment letter, 
we realized that there is no practical reason to maintain that 
distinction.
    We also realized that there was no reason to maintain the guidance 
in the prior and proposed rules that required the use of listing 1.03 
when there had been reconstructive surgery. Final listing 14.09A1 is 
sufficient to cover the situation described in listing 1.03 for 
individuals with inflammatory arthritis who have had reconstructive 
surgery of a major peripheral weight-bearing joint and have been unable 
to ambulate effectively for at least 12 months or can be expected to be 
unable to ambulate effectively for at least 12 months.
    As already noted in the summary of the changes in these rules, we 
revised the second sentence in 1.00B1, in the introductory text of the 
musculoskeletal system listings, to reflect these changes. We also made 
corresponding changes in part B of the listings, in 101.00B, 114.00D6, 
and 114.09A.
    Comment: One commenter suggested that the term ``dorsolumbar'' 
ankylosis in proposed listing 14.09C should indicate that 
``dorsolumbar'' means dorsal and lumbar, not either one.
    Response: We did not adopt the comment. The term ``dorsolumbar'' is 
a common medical term that is generally recognized to mean the area of 
the spine relating to the lower thoracic and upper lumbar vertebral 
region of the back. We used this term in prior listing 14.09B2 (final 
listing 14.09C1), and we are not aware that it caused any confusion. 
However, we will reinforce the definition when we conduct training on 
these final rules.

Other Disorders

    Comment: One commenter noted that in proposed 14.00D6c(v) we 
mentioned Lyme disease only by name and only as an impairment that we 
evaluate under listing 14.09 for inflammatory arthritis. The commenter 
said that the symptoms of Lyme disease are the same as for SLE, and 
suggested that we provide criteria for evaluating the disorder similar 
to the criteria for SLE and Sj[ouml]gren's syndrome. The commenter also 
noted that Lyme disease with co-infections can be fatal.
    This commenter and a second commenter noted that, like other immune 
system disorders, the symptoms of Lyme disease can be ``invisible,'' 
making it difficult to evaluate disability. One of the commenters 
suggested that we should not focus on the name of the disease but on 
its effects and made recommendations for how we could better adjudicate 
cases; for example, by giving more weight to reports from treating 
physicians. This commenter also noted that the symptoms of the 
impairment can improve at times but that we should not assume that an 
individual is not disabled just because he or she is able to function 
well for a short period. Both commenters also described difficulties in 
our adjudication system.
    Response: We agree with the commenters that some individuals with 
Lyme disease have symptoms that are the same as or similar to the 
symptoms of SLE, Sj[ouml]gren's syndrome, and other immune system 
disorders we include in these listings. However, there are hundreds of 
disorders that affect the immune system, and we are not able to list 
all of them in our listing of impairments. In proposed (now final) 
14.00D6c, we included Lyme disease as an example of a disorder that 
could cause inflammatory arthritis because that is the most frequent 
disabling outcome of Lyme disease.
    Some individuals with disabling Lyme disease will have impairments 
that meet the requirements of final listings 14.09B and especially 
14.09D. Final listing 14.09D recognizes that individuals with Lyme 
disease and other disorders that can cause inflammatory arthritis can 
have serious functional limitations as a result of their symptoms, 
including the kinds of symptoms described by the commenters. The 
functional criteria in final listing 14.09D and throughout the final 
immune system disorders listings recognize the ``invisible'' nature of 
most immune system disorders. As we noted in the preamble to the NPRM, 
they also consider the variable nature of the symptoms of immune system 
disorders. (See 71 FR at 44441)
    As in the proposed rule, final 14.00J also provides that 
individuals with immune system disorders that do not meet the criteria 
of one of these listings can have impairments resulting from their 
immune system disorders that meet the requirements of listings in other 
body systems, such as neurological or mental disorders. In final 
14.00D6e(iii), as in the NPRM, we list such extra-articular features of 
immune disorders that can cause inflammatory arthritis by body system 
to provide guidance about such other effects that these disorders, 
including Lyme disease, may have. However, in reviewing these comment 
letters and the proposed rules, we realized that we had inadvertently 
omitted reference to possible mental signs and symptoms in this 
section. Therefore, we are including the phrase ``mental (cognitive 
dysfunction, poor memory)'' in final 14.00D6e(iii) in response to these 
comments. The phrase is the same one that we use in final 14.00D7a(ii) 
for Sj[ouml]gren's syndrome. We also added the same language in final 
114.00D6e(iii) in part B.
    Individuals who have Lyme disease but who do not have repeated 
manifestations of inflammatory arthritis can also qualify under the 
listings for SLE, Sj[ouml]gren's syndrome, or other appropriate 
listings in the immune disorders body system or any other appropriate 
body system based on our policy of medical equivalence.
    Finally, we carefully considered the recommendations of the 
commenter

[[Page 14599]]

who suggested ways to improve our evaluations of cases involving Lyme 
disease. These suggestions were covered by other general regulations 
and policy statements we have, such as our policies for evaluating 
symptoms and treating source opinions. Therefore, we decided not to 
adopt those comments.
    Comment: Several commenters suggested that we add additional 
disorders to the listings, including myasthenia gravis, multiple 
sclerosis, colon cancer, chronic fatigue syndrome, and fibromyalgia.
    Response: We have not added the specific disorders suggested by the 
commenters. In some instances the disorders are already included in our 
rules:
     Multiple sclerosis, listing 11.09 (neurological body 
system),
     Myasthenia gravis, listing 11.12 (neurological body 
system), and
     Stage IV colon cancer, listing 13.18 (malignant neoplastic 
diseases body system).

You can see all of our listings at: http://www.socialsecurity.gov/OP_Home/cfr20/404/404-ap10.htm and http://www.socialsecurity.gov/disability/professionals/bluebook/index.htm.
    In other instances, such as fibromyalgia and chronic fatigue 
syndrome, we did not add the suggested disorders. Although we recognize 
fibromyalgia and chronic fatigue syndrome as medically determinable 
impairments, we do not list them, in part because there is not 
sufficient agreement in the medical community about the nature of these 
impairments. However, we may find that fibromyalgia and chronic fatigue 
syndrome medically equal a listing or that they are disabling at a 
later step of the sequential evaluation process for adults or children. 
See, for example, Social Security Ruling (SSR) 99-2p, Titles II and 
XVI: Evaluating Cases Involving Chronic Fatigue Syndrome (CFS), 64 FR 
23380 (1999), available at http://www.socialsecurity.gov/OP_Home/rulings/di/01/SSR99-02-di-01.html.
    Comment: Several commenters who have multiple immune disorders or 
family members with immune disorders noted that having multiple immune 
system disorders can significantly limit an individual's ability to 
function and to work. One commenter suggested that we include other 
autoimmune diseases that affect only one organ, such as Hashimoto's or 
Graves disease, as an additional disease entity to support one of the 
other listed immune system disorders in a disability claim.
    Response: We agree that an individual with multiple immune system 
disorders may have significant limitations in the ability to function. 
However, we did not adopt this comment because we believe that the new 
functional criteria in each of the final listings will help individuals 
like the commenters and their family members without additional changes 
to the listings.

Other Comments

    Comment: One commenter addressed our proposal to change the 
requirement throughout the listings in this body system that an 
individual have all four of the constitutional symptoms and signs to a 
requirement for only two of the constitutional symptoms and signs. The 
commenter noted that fatigue and malaise are both symptoms, and 
therefore, that an individual could meet this requirement of several of 
the immune system disorders listings with two symptoms. The commenter 
also indicated that these symptoms are ``exceedingly common'' in the 
general population and said that they are poor discriminators of 
severity. Therefore, the commenter suggested that we consider fatigue 
and malaise as one criterion, that is, fatigue/malaise, rather than two 
separate criteria.
    Response: We did not adopt this comment. As we define them in final 
14.00C2 and 114.00C2, the symptoms of fatigue and malaise are quite 
severe and not at all common in the general population. As we indicated 
in the preamble to the NPRM, we proposed to add these definitions ``in 
response to the many comments we received [on the ANPRM and in the 
outreach meetings] that indicated that the fatigue and malaise that 
people who have immune system disorders experience can be very 
limiting.'' (71 FR at 44435) In discussing the proposed functional 
criteria, we also reported that ``[a] number of people indicated that 
the fatigue associated with these disorders was not merely a feeling of 
tiredness but a more profound and debilitating experience.'' (71 FR at 
44440) This is consistent with information we received from medical 
specialists in immune system disorders at the outreach meetings and our 
own review of the medical literature. (See 71 FR at 44448 for a list of 
the medical references we consulted when we were preparing the proposed 
rules.) Moreover, the presence of two of the constitutional symptoms 
and signs is only one criterion in the listings. To meet any of the 
listings that include this criterion, the individual must also have an 
established immune system disorder and involvement of at least two 
organs or body systems. As we explained in the preamble to the NPRM, we 
proposed to revise the requirement for all four constitutional symptoms 
and signs to ``at least two'' of the constitutional symptoms or signs:

because we believe that the requirement in the current listing is 
too severe. We believe that any individual with an autoimmune 
disorder involving two or more organs/body systems with one organ/
body system involved to at least a moderate level of severity and 
who has at least two of the constitutional symptoms and signs in 
these listings will have an impairment that precludes any gainful 
activity.

(71 FR at 44442)
    Comment: One commenter noted that multiple listings (for example, 
proposed listings 14.02B, 14.03B, and 14.06B) used the phrase ``without 
the requisite findings in A.'' The commenter thought that the phrase 
was unclear, and that it was not clear when this listing criterion 
would apply. For example, the commenter asked whether this meant in 
proposed listing 14.02B that the individual had involvement of only one 
organ or that there was involvement of two organs but neither to a 
``moderate'' degree.
    Response: We adopted the comment by deleting the phrase ``but 
without the requisite findings in'' from the proposed listings that 
included that phrase, except in listings 14.08K and 114.08L. Because of 
their structure, some proposed listings referred only to paragraph A, 
while others referred to additional paragraphs. For example, proposed 
listing 14.04D included the phrase ``but without the requisite findings 
in A, B, or C.'' We removed all of these references. We also made 
conforming editorial changes to the first sentence in final 14.00I1 and 
114.00I1.
    In considering the comment, we realized that the phrase was 
unnecessary and that deleting it would not change our intent. For 
example, an individual's SLE meets final listing 14.02A if there is 
involvement of at least two organs/body systems with one of the organs/
body systems involved to at least a moderate level of severity and with 
at least two of the constitutional symptoms and signs. An individual's 
SLE meets listing 14.02B if it causes repeated manifestations of SLE, 
at least two of the constitutional symptoms and signs, and a ``marked'' 
limitation in one of the listed areas of functioning. There is no need 
for a reference to listing 14.02A in listing 14.02B.
    The same can be said about final listings 14.08K and 114.08L. 
However, we decided to keep the phrase in those listings because it has 
been in the prior versions of those listings for many years, is clear 
in the context of those listings, and is followed by parenthetical

[[Page 14600]]

examples that we do not want to remove.
    We also realized that related language we proposed in the listings 
was unclear in other ways. The phrase ``Repeated manifestations of [the 
listed immune disorder] * * * resulting in at least two of the 
constitutional symptoms or signs'' could have been misinterpreted. It 
could have been read to mean that we would need evidence demonstrating 
that the constitutional symptoms or signs were the result of the 
manifestations of the immune system disorder, not the immune system 
disorder itself. We revised the language to clarify our intent, which 
is that the constitutional symptoms and signs can be the result of 
either the immune disorder itself or any of its manifestations. Also, 
some of the listings, for example, proposed listing 14.02A2 (which was 
referenced by proposed listing 14.02B), used the unclear phrase ``At 
least two of the following constitutional symptoms or signs: Severe 
fatigue, fever, malaise, or involuntary weight loss.'' (Emphasis 
added.) This could have been misinterpreted to mean that there are 
other constitutional symptoms and signs. Therefore, we revised all of 
the listings that included this statement to say ``At least two of the 
constitutional symptoms or signs (severe fatigue, fever, malaise, or 
involuntary weight loss).'' For consistency with this change, we also 
revised our definition of ``constitutional symptoms or signs'' in 
proposed 14.00C and 114.00C to explain that the fatigue must be 
``severe fatigue'' for purposes of these listings. This is not a 
substantive change in the proposed rules because in fact all of the 
proposed listings required ``severe fatigue'' when they referred to 
constitutional symptoms or signs.
    Comment: One commenter suggested that we specify in these rules 
which tests we will not purchase, such as angiography and tissue 
biopsy. The commenter noted that this would also make the immune system 
disorders listings consistent with the most recent revision of the 
cardiovascular system listings, which we issued in early 2006.
    Response: We adopted the comment. The new guidance is in final 
14.00D2b, 14.00D4b, and 14.00F1 and the corresponding childhood 
sections. We considered adding the same language in final 14.00F3 and 
114.00F3 but decided not to because there are some manifestations for 
which we may purchase tests, such as routine types of blood tests.
    Comment: One commenter noted that the heading in proposed 14.00D 
was different than the headings in proposed 14.00E and 14.00F. The 
commenter suggested revisions to the headings of 14.00D, 14.00E, and 
14.00F that would make them consistent with each other.
    Response: We adopted the comment. The commenter recommended that we 
change the headings to declarative statements, but we retained the 
question form to be consistent with most of the other headings in this 
body system. Otherwise, we used the same language the commenter 
recommended.
    Comment: One commenter suggested that we use simple terms in these 
rules.
    Response: We have simplified the language as much as we can given 
the complexity of these disorders. However, to provide useful 
adjudicative guidance, our rules need to reference the technical terms 
that are used in medical records and severity terms we use in our 
regulations. When appropriate, we have provided definitions of these 
terms in final 14.00C and 114.00C and elsewhere in these final rules.
    Comment: One commenter questioned how we can give benefits to some 
and deny others when an autoimmune disease is a disabling disease with 
no hope of getting better.
    Response: While we understand the concern of the commenter, we also 
recognize that many individuals who are diagnosed with autoimmune 
disorders lead reasonably normal lives, including regular employment. 
We can pay benefits only to individuals who are under a disability as 
defined in the Act and in our regulations. The issue in a disability 
determination under the listings is whether the individual has an 
impairment that prevents him or her from engaging in any gainful 
activity (or in a child, that causes ``marked and severe functional 
limitations''), and that can be expected to result in death or which 
has lasted or can be expected to last for a continuous period of not 
less than 12 months. If the impairment does not meet or medically equal 
the listings, we may still find that the impairment is disabling based 
on an assessment of the individual's residual functional capacity (or 
the child's ability to function).
    Comment: One commenter suggested that it will be essential to 
provide a training program for all workers who are involved in the 
disability process, particularly those who make the initial 
determination. The commenter indicated that it will be necessary for 
adjudicators to understand all of the information in the introductory 
text and that this will be difficult for them. The commenter also 
remarked that we should be aware that it will be more burdensome and 
time-consuming for treating physicians to understand the nuances of 
these rules and that physicians have less and less time to deal with 
extensive reading in order to complete a form or to write letters for 
their patients' disability claims.
    Response: We agree that training on these final rules will be 
needed. We will conduct training that will provide adjudicators with 
guidance on applying these listings.
    We do not believe the expanded guidance in these final rules 
imposes additional burdens on treating physicians. It is our 
responsibility to decide whether individuals meet the criteria of these 
rules, and the information we need from treating sources so that we can 
make our decision is no different under these rules than it was before. 
As we have already explained, we expect that in some cases we will need 
even less information than we did in the past because of additional 
medical and functional criteria in these listings that will permit us 
to allow individuals who should be allowed under the listings instead 
of at a later step in the sequential evaluation process.
    Even the new functional criteria in the listings will not impose a 
new burden on treating sources. This is because when we ask for 
information from treating and other medical sources we also ask them 
for opinions about how their patients' medical conditions limit 
functioning in case we need to consider residual functional capacity 
or, for children, functional equivalence. See, for example, Sec. Sec.  
404.1513 and 416.913 of our regulations. We will be able to use the 
same information treating sources provide for residual functional 
capacity assessments or determinations about functional equivalence to 
make our determinations about limitations under the new listings and, 
in some cases, will need even less information when the functional 
limitations are clearly as serious as the listings describe.

What is our authority to make rules and set procedures for determining 
whether a person is disabled under the statutory definition?

    Section 205(a) of the Act and, by reference to section 205(a), 
section 1631(d)(1) provide that:

    The Commissioner of Social Security shall have full power and 
authority to make rules and regulations and to establish procedures, 
not inconsistent with the provisions of this title, which are 
necessary or appropriate to carry out such provisions, and shall 
adopt reasonable and proper rules and regulations to regulate and 
provide for the nature and extent of the proofs and evidence and the 
method of taking and furnishing the same in order to establish the 
right to benefits hereunder.

[[Page 14601]]

Regulatory Procedures

Executive Order 12866

    We have consulted with the Office of Management and Budget (OMB) 
and determined that these final rules meet the requirements for a 
significant regulatory action under Executive Order 12866, as amended. 
Thus, they were subject to OMB review.

Regulatory Flexibility Act

    We certify that these final rules do not have a significant 
economic impact on a substantial number of small entities because they 
affect only individuals. Thus, a regulatory flexibility analysis as 
provided in the Regulatory Flexibility Act, as amended, is not 
required.

Paperwork Reduction Act

    The Paperwork Reduction Act (PRA) of 1995 says that no persons are 
required to respond to a collection of information unless it displays a 
valid OMB control number. In accordance with the PRA, SSA is providing 
notice that OMB has approved the information collection requirements 
contained in sections 14.00B, 14.00D, 14.00E, 14.00F, 114.00B, 114.00D, 
114.00E, 114.00F, 114.08 and 114.09 of these final rules. The OMB 
Control Number for this collection is 0960-0642, expiring March 31, 
2008.

(Catalog of Federal Domestic Assistance Program Nos. 96.001, Social 
Security-Disability Insurance; 96.002, Social Security-Retirement 
Insurance; 96.004, Social Security-Survivors Insurance; and 96.006, 
Supplemental Security Income)

List of Subjects in 20 CFR Part 404

    Administrative practice and procedure, Blind, Disability benefits, 
Old-Age, Survivors, and Disability Insurance, Reporting and 
recordkeeping requirements, Social Security.

Michael J. Astrue,
Commissioner of Social Security.

0
For the reasons set out in the preamble, we are amending subpart P of 
part 404 of chapter III of title 20 of the Code of Federal Regulations 
as set forth below:

PART 404--FEDERAL OLD-AGE, SURVIVORS AND DISABILITY INSURANCE 
(1950-)

0
1. The authority citation for subpart P of part 404 continues to read 
as follows:

    Authority: Secs. 202, 205(a), (b), and (d)-(h), 216(i), 221(a) 
and (i), 222(c), 223, 225, and 702(a)(5) of the Social Security Act 
(42 U.S.C. 402, 405(a), (b), and (d)-(h), 416(i), 421(a) and (i), 
422(c), 423, 425, and 902(a)(5)); sec. 211(b), Pub. L. 104-193, 110 
Stat. 2105, 2189; sec. 202, Pub. L. 108-203, 118 Stat. 509 (42 
U.S.C. 902 note).

Appendix 1 to Subpart P of Part 404--[Amended]

0
2. Appendix 1 to subpart P of Part 404 is amended as follows:
0
a. Revise the body system name and the expiration date in item 15 of 
the introductory text before part A of appendix 1.
0
b. Amend the table of contents for part A of appendix 1 by revising the 
body system name for section 14.00.
0
c. Revise the second sentence of section 1.00B1 of part A of appendix 
1.
0
d. Revise the fourth sentence of section 1.00L of part A of appendix 1.
0
e. Revise section 8.00D3 of part A of appendix 1.
0
f. Revise the second sentence of section 13.00A of part A of appendix 
1.
0
g. Revise section 14.00 of part A of appendix 1.
0
h. Amend the table of contents for part B of appendix 1 by revising the 
body system name for section 14.00.
0
i. Revise the second sentence of section 101.00B1 of part B of appendix 
1.
0
j. Revise the fourth sentence of section 101.00L of part B of appendix 
1.
0
k. Revise section 108.00D3 of part B of appendix 1.
0
l. Revise the second sentence of section 113.00A of part B of appendix 
1.
0
m. Revise section 114.00 of part B of appendix 1.
    The revised text is set forth as follows:

Appendix 1 to Subpart P of Part 404--Listing of Impairments

* * * * *
    15. Immune System Disorders (14.00 and 114.00): June 16, 2016.

Part A

* * * * *
    14.00 Immune System Disorders.

1.00 MUSCULOSKELETAL SYSTEM

* * * * *
    B. * * *
    1 * * * The provisions of 1.02 and 1.03 notwithstanding, 
inflammatory arthritis is evaluated under 14.09 (see 14.00D6). * * *
* * * * *
    L. * * * When the abnormal curvature of the spine results in 
symptoms related to fixation of the dorsolumbar or cervical spine, 
evaluation of equivalence may be made by reference to 14.09C. * * *
* * * * *

8.00 SKIN DISORDERS

* * * * *
    D. * * *
    3. Autoimmune disorders and other immune system disorders (for 
example, systemic lupus erythematosus (SLE), scleroderma, human 
immunodeficiency virus (HIV) infection, and Sjogren's syndrome) 
often involve more than one body system. We first evaluate these 
disorders under the immune system disorders listings in 14.00. We 
evaluate SLE under 14.02, scleroderma under 14.04, HIV infection 
under 14.08, and Sjogren's syndrome under 14.10.
* * * * *

13.00 MALIGNANT NEOPLASTIC DISEASES

    A. * * * We use the criteria in 14.08E to evaluate carcinoma of 
the cervix, Kaposi's sarcoma, lymphoma, and squamous cell carcinoma 
of the anal canal and anal margin if you also have HIV infection.
* * * * *

14.00 IMMUNE SYSTEM DISORDERS

    A. What disorders do we evaluate under the immune system 
disorders listings?
    1. We evaluate immune system disorders that cause dysfunction in 
one or more components of your immune system.
    a. The dysfunction may be due to problems in antibody 
production, impaired cell-mediated immunity, a combined type of 
antibody/cellular deficiency, impaired phagocytosis, or complement 
deficiency.
    b. Immune system disorders may result in recurrent and unusual 
infections, or inflammation and dysfunction of the body's own 
tissues. Immune system disorders can cause a deficit in a single 
organ or body system that results in extreme (that is, very serious) 
loss of function. They can also cause lesser degrees of limitations 
in two or more organs or body systems, and when associated with 
symptoms or signs, such as severe fatigue, fever, malaise, diffuse 
musculoskeletal pain, or involuntary weight loss, can also result in 
extreme limitation.
    c. We organize the discussions of immune system disorders in 
three categories: Autoimmune disorders; Immune deficiency disorders, 
excluding human immunodeficiency virus (HIV) infection; and HIV 
infection.
    2. Autoimmune disorders (14.00D). Autoimmune disorders are 
caused by dysfunctional immune responses directed against the body's 
own tissues, resulting in chronic, multisystem impairments that 
differ in clinical manifestations, course, and outcome. They are 
sometimes referred to as rheumatic diseases, connective tissue 
disorders, or collagen vascular disorders. Some of the features of 
autoimmune disorders in adults differ from the features of the same 
disorders in children.
    3. Immune deficiency disorders, excluding HIV infection 
(14.00E). Immune deficiency disorders are characterized by recurrent 
or unusual infections that respond poorly to treatment, and are 
often associated with complications affecting other parts of the 
body. Immune deficiency disorders are classified as either primary 
(congenital) or acquired. Individuals with immune deficiency 
disorders also have an increased risk of malignancies and of having 
autoimmune disorders.
    4. Human immunodeficiency virus (HIV) infection (14.00F). HIV 
infection may be characterized by increased susceptibility to 
opportunistic infections, cancers, or other conditions, as described 
in 14.08.
    B. What information do we need to show that you have an immune 
system disorder?

[[Page 14602]]

Generally, we need your medical history, a report(s) of a physical 
examination, a report(s) of laboratory findings, and in some 
instances, appropriate medically acceptable imaging or tissue biopsy 
reports to show that you have an immune system disorder. Therefore, 
we will make every reasonable effort to obtain your medical history, 
medical findings, and results of laboratory tests. We explain the 
information we need in more detail in the sections below.

C. Definitions

    1. Appropriate medically acceptable imaging includes, but is not 
limited to, angiography, x-ray imaging, computerized axial 
tomography (CAT scan) or magnetic resonance imaging (MRI), with or 
without contrast material, myelography, and radionuclear bone scans. 
``Appropriate'' means that the technique used is the proper one to 
support the evaluation and diagnosis of the impairment.
    2. Constitutional symptoms or signs, as used in these listings, 
means severe fatigue, fever, malaise, or involuntary weight loss. 
Severe fatigue means a frequent sense of exhaustion that results in 
significantly reduced physical activity or mental function. Malaise 
means frequent feelings of illness, bodily discomfort, or lack of 
well-being that result in significantly reduced physical activity or 
mental function.
    3. Disseminated means that a condition is spread over a 
considerable area. The type and extent of the spread will depend on 
your specific disease.
    4. Dysfunction means that one or more of the body regulatory 
mechanisms are impaired, causing either an excess or deficiency of 
immunocompetent cells or their products.
    5. Extra-articular means ``other than the joints''; for example, 
an organ(s) such as the heart, lungs, kidneys, or skin.
    6. Inability to ambulate effectively has the same meaning as in 
1.00B2b.
    7. Inability to perform fine and gross movements effectively has 
the same meaning as in 1.00B2c.
    8. Major peripheral joints has the same meaning as in 1.00F.
    9. Persistent means that a sign(s) or symptom(s) has continued 
over time. The precise meaning will depend on the specific immune 
system disorder, the usual course of the disorder, and the other 
circumstances of your clinical course.
    10. Recurrent means that a condition that previously responded 
adequately to an appropriate course of treatment returns after a 
period of remission or regression. The precise meaning, such as the 
extent of response or remission and the time periods involved, will 
depend on the specific disease or condition you have, the body 
system affected, the usual course of the disorder and its treatment, 
and the other facts of your particular case.
    11. Resistant to treatment means that a condition did not 
respond adequately to an appropriate course of treatment. Whether a 
response is adequate or a course of treatment is appropriate will 
depend on the specific disease or condition you have, the body 
system affected, the usual course of the disorder and its treatment, 
and the other facts of your particular case.
    12. Severe means medical severity as used by the medical 
community. The term does not have the same meaning as it does when 
we use it in connection with a finding at the second step of the 
sequential evaluation processes in Sec. Sec.  404.1520, 416.920, and 
416.924.

D. How do we document and evaluate the listed autoimmune disorders?

    1. Systemic lupus erythematosus (14.02).
    a. General. Systemic lupus erythematosus (SLE) is a chronic 
inflammatory disease that can affect any organ or body system. It is 
frequently, but not always, accompanied by constitutional symptoms 
or signs (severe fatigue, fever, malaise, involuntary weight loss). 
Major organ or body system involvement can include: Respiratory 
(pleuritis, pneumonitis), cardiovascular (endocarditis, myocarditis, 
pericarditis, vasculitis), renal (glomerulonephritis), hematologic 
(anemia, leukopenia, thrombocytopenia), skin (photosensitivity), 
neurologic (seizures), mental (anxiety, fluctuating cognition 
(``lupus fog''), mood disorders, organic brain syndrome, psychosis), 
or immune system disorders (inflammatory arthritis). 
Immunologically, there is an array of circulating serum auto-
antibodies and pro- and anti-coagulant proteins that may occur in a 
highly variable pattern.
    b. Documentation of SLE. Generally, but not always, the medical 
evidence will show that your SLE satisfies the criteria in the 
current ``Criteria for the Classification of Systemic Lupus 
Erythematosus'' by the American College of Rheumatology found in the 
most recent edition of the Primer on the Rheumatic Diseases 
published by the Arthritis Foundation.
    2. Systemic vasculitis (14.03).
    a. General.
    (i) Vasculitis is an inflammation of blood vessels. It may occur 
acutely in association with adverse drug reactions, certain chronic 
infections, and occasionally, malignancies. More often, it is 
chronic and the cause is unknown. Symptoms vary depending on which 
blood vessels are involved. Systemic vasculitis may also be 
associated with other autoimmune disorders; for example, SLE or 
dermatomyositis.
    (ii) There are several clinical patterns, including but not 
limited to polyarteritis nodosa, Takayasu's arteritis (aortic arch 
arteritis), giant cell arteritis (temporal arteritis), and Wegener's 
granulomatosis.
    b. Documentation of systemic vasculitis. Angiography or tissue 
biopsy confirms a diagnosis of systemic vasculitis when the disease 
is suspected clinically. When you have had angiography or tissue 
biopsy for systemic vasculitis, we will make every reasonable effort 
to obtain reports of the results of that procedure. However, we will 
not purchase angiography or tissue biopsy.
    3. Systemic sclerosis (scleroderma) (14.04).
    a. General. Systemic sclerosis (scleroderma) constitutes a 
spectrum of disease in which thickening of the skin is the clinical 
hallmark. Raynaud's phenomenon, often medically severe and 
progressive, is present frequently and may be the peripheral 
manifestation of a vasospastic abnormality in the heart, lungs, and 
kidneys. The CREST syndrome (calcinosis, Raynaud's phenomenon, 
esophageal dysmotility, sclerodactyly, and telangiectasia) is a 
variant that may slowly progress over years to the generalized 
process, systemic sclerosis.
    b. Diffuse cutaneous systemic sclerosis. In diffuse cutaneous 
systemic sclerosis (also known as diffuse scleroderma), major organ 
or systemic involvement can include the gastrointestinal tract, 
lungs, heart, kidneys, and muscle in addition to skin or blood 
vessels. Although arthritis can occur, joint dysfunction results 
primarily from soft tissue/cutaneous thickening, fibrosis, and 
contractures.
    c. Localized scleroderma (linear scleroderma and morphea).
    (i) Localized scleroderma (linear scleroderma and morphea) is 
more common in children than in adults. However, this type of 
scleroderma can persist into adulthood. To assess the severity of 
the impairment, we need a description of the extent of involvement 
of linear scleroderma and the location of the lesions. For example, 
linear scleroderma involving the arm but not crossing any joints is 
not as functionally limiting as sclerodactyly (scleroderma localized 
to the fingers). Linear scleroderma of a lower extremity involving 
skin thickening and atrophy of underlying muscle or bone can result 
in contractures and leg length discrepancy. In such cases, we may 
evaluate your impairment under the musculoskeletal listings (1.00).
    (ii) When there is isolated morphea of the face causing facial 
disfigurement from unilateral hypoplasia of the mandible, maxilla, 
zygoma, or orbit, adjudication may be more appropriate under the 
criteria in the affected body system, such as special senses and 
speech (2.00) or mental disorders (12.00).
    (iii) Chronic variants of these syndromes include disseminated 
morphea, Shulman's disease (diffuse fasciitis with eosinophilia), 
and eosinophilia-myalgia syndrome (often associated with toxins such 
as toxic oil or contaminated tryptophan), all of which can impose 
medically severe musculoskeletal dysfunction and may also lead to 
restrictive pulmonary disease. We evaluate these variants of the 
disease under the criteria in the musculoskeletal listings (1.00) or 
respiratory system listings (3.00).
    d. Documentation of systemic sclerosis (scleroderma). 
Documentation involves differentiating the clinical features of 
systemic sclerosis (scleroderma) from other autoimmune disorders. 
However, there may be an overlap.
    4. Polymyositis and dermatomyositis (14.05).
    a. General. Polymyositis and dermatomyositis are related 
disorders that are characterized by an inflammatory process in 
striated muscle, occurring alone or in association with other 
autoimmune disorders or malignancy. The most common manifestations 
are symmetric weakness, and less frequently, pain and tenderness of 
the proximal limb-girdle (shoulder or pelvic) musculature. There may 
also be involvement of the cervical, cricopharyngeal, esophageal, 
intercostal, and diaphragmatic muscles.

[[Page 14603]]

    b. Documentation of polymyositis and dermatomyositis. Generally, 
but not always, polymyositis is associated with elevated serum 
muscle enzymes (creatine phosphokinase (CPK), aminotransferases, and 
aldolase), and characteristic abnormalities on electromyography and 
muscle biopsy. In dermatomyositis there are characteristic skin 
findings in addition to the findings of polymyositis. When you have 
had electromyography or muscle biopsy for polymyositis or 
dermatomyositis, we will make every reasonable effort to obtain 
reports of the results of that procedure. However, we will not 
purchase electromyography or muscle biopsy.
    c. Additional information about how we evaluate polymyositis and 
dermatomyositis under the listings.
    (i) Weakness of your pelvic girdle muscles that results in your 
inability to rise independently from a squatting or sitting position 
or to climb stairs may be an indication that you are unable to 
ambulate effectively. Weakness of your shoulder girdle muscles may 
result in your inability to perform lifting, carrying, and reaching 
overhead, and also may seriously affect your ability to perform 
activities requiring fine movements. We evaluate these limitations 
under 14.05A.
    (ii) We use the malignant neoplastic diseases listings (13.00) 
to evaluate malignancies associated with polymyositis or 
dermatomyositis. We evaluate the involvement of other organs/body 
systems under the criteria for the listings in the affected body 
system.
    5. Undifferentiated and mixed connective tissue disease (14.06).
    a. General. This listing includes syndromes with clinical and 
immunologic features of several autoimmune disorders, but which do 
not satisfy the criteria for any of the specific disorders 
described. For example, you may have clinical features of SLE and 
systemic vasculitis, and the serologic (blood test) findings of 
rheumatoid arthritis.
    b. Documentation of undifferentiated and mixed connective tissue 
disease. Undifferentiated connective tissue disease is diagnosed 
when clinical features and serologic (blood test) findings, such as 
rheumatoid factor or antinuclear antibody (consistent with an 
autoimmune disorder) are present but do not satisfy the criteria for 
a specific disease. Mixed connective tissue disease (MCTD) is 
diagnosed when clinical features and serologic findings of two or 
more autoimmune diseases overlap.
    6. Inflammatory arthritis (14.09).
    a. General. The spectrum of inflammatory arthritis includes a 
vast array of disorders that differ in cause, course, and outcome. 
Clinically, inflammation of major peripheral joints may be the 
dominant manifestation causing difficulties with ambulation or fine 
and gross movements; there may be joint pain, swelling, and 
tenderness. The arthritis may affect other joints, or cause less 
limitation in ambulation or the performance of fine and gross 
movements. However, in combination with extra-articular features, 
including constitutional symptoms or signs (severe fatigue, fever, 
malaise, involuntary weight loss), inflammatory arthritis may result 
in an extreme limitation.
    b. Inflammatory arthritis involving the axial spine 
(spondyloarthropathy). In adults, inflammatory arthritis involving 
the axial spine may be associated with disorders such as:
    (i) Reiter's syndrome;
    (ii) Ankylosing spondylitis;
    (iii) Psoriatic arthritis;
    (iv) Whipple's disease;
    (v) Beh[ccedil]et's disease; and
    (vi) Inflammatory bowel disease.
    c. Inflammatory arthritis involving the peripheral joints. In 
adults, inflammatory arthritis involving peripheral joints may be 
associated with disorders such as:
    (i) Rheumatoid arthritis;
    (ii) Sj[ouml]gren's syndrome;
    (iii) Psoriatic arthritis;
    (iv) Crystal deposition disorders (gout and pseudogout);
    (v) Lyme disease; and
    (vi) Inflammatory bowel disease.
    d. Documentation of inflammatory arthritis. Generally, but not 
always, the diagnosis of inflammatory arthritis is based on the 
clinical features and serologic findings described in the most 
recent edition of the Primer on the Rheumatic Diseases published by 
the Arthritis Foundation.
    e. How we evaluate inflammatory arthritis under the listings.
    (i) Listing-level severity in 14.09A and 14.09C1 is shown by an 
impairment that results in an ``extreme'' (very serious) limitation. 
In 14.09A, the criterion is satisfied with persistent inflammation 
or deformity in one major peripheral weight-bearing joint resulting 
in the inability to ambulate effectively (as defined in 14.00C6) or 
one major peripheral joint in each upper extremity resulting in the 
inability to perform fine and gross movements effectively (as 
defined in 14.00C7). In 14.09C1, if you have the required ankylosis 
(fixation) of your cervical or dorsolumbar spine, we will find that 
you have an extreme limitation in your ability to see in front of 
you, above you, and to the side. Therefore, inability to ambulate 
effectively is implicit in 14.09C1, even though you might not 
require bilateral upper limb assistance.
    (ii) Listing-level severity is shown in 14.09B, 14.09C2, and 
14.09D by inflammatory arthritis that involves various combinations 
of complications of one or more major peripheral joints or other 
joints, such as inflammation or deformity, extra-articular features, 
repeated manifestations, and constitutional symptoms or signs. 
Extra-articular impairments may also meet listings in other body 
systems.
    (iii) Extra-articular features of inflammatory arthritis may 
involve any body system; for example: Musculoskeletal (heel 
enthesopathy), ophthalmologic (iridocyclitis, keratoconjunctivitis 
sicca, uveitis), pulmonary (pleuritis, pulmonary fibrosis or 
nodules, restrictive lung disease), cardiovascular (aortic valve 
insufficiency, arrhythmias, coronary arteritis, myocarditis, 
pericarditis, Raynaud's phenomenon, systemic vasculitis), renal 
(amyloidosis of the kidney), hematologic (chronic anemia, 
thrombocytopenia), neurologic (peripheral neuropathy, radiculopathy, 
spinal cord or cauda equina compression with sensory and motor 
loss), mental (cognitive dysfunction, poor memory), and immune 
system (Felty's syndrome (hypersplenism with compromised immune 
competence)).
    (iv) If both inflammation and chronic deformities are present, 
we evaluate your impairment under the criteria of any appropriate 
listing.
    7. Sj[ouml]gren's syndrome (14.10).
    a. General.
    (i) Sj[ouml]gren's syndrome is an immune-mediated disorder of 
the exocrine glands. Involvement of the lacrimal and salivary glands 
is the hallmark feature, resulting in symptoms of dry eyes and dry 
mouth, and possible complications, such as corneal damage, 
blepharitis (eyelid inflammation), dysphagia (difficulty in 
swallowing), dental caries, and the inability to speak for extended 
periods of time. Involvement of the exocrine glands of the upper 
airways may result in persistent dry cough.
    (ii) Many other organ systems may be involved, including 
musculoskeletal (arthritis, myositis), respiratory (interstitial 
fibrosis), gastrointestinal (dysmotility, dysphagia, involuntary 
weight loss), genitourinary (interstitial cystitis, renal tubular 
acidosis), skin (purpura, vasculitis), neurologic (central nervous 
system disorders, cranial and peripheral neuropathies), mental 
(cognitive dysfunction, poor memory), and neoplastic (lymphoma). 
Severe fatigue and malaise are frequently reported. Sj[ouml]gren's 
syndrome may be associated with other autoimmune disorders (for 
example, rheumatoid arthritis or SLE); usually the clinical features 
of the associated disorder predominate.
    b. Documentation of Sj[ouml]gren's syndrome. If you have 
Sj[ouml]gren's syndrome, the medical evidence will generally, but 
not always, show that your disease satisfies the criteria in the 
current ``Criteria for the Classification of Sj[ouml]gren's 
Syndrome'' by the American College of Rheumatology found in the most 
recent edition of the Primer on the Rheumatic Diseases published by 
the Arthritis Foundation.

E. How do we document and evaluate immune deficiency disorders, 
excluding HIV infection?

    1. General.
    a. Immune deficiency disorders can be classified as:
    (i) Primary (congenital); for example, X-linked 
agammaglobulinemia, thymic hypoplasia (DiGeorge syndrome), severe 
combined immunodeficiency (SCID), chronic granulomatous disease 
(CGD), C1 esterase inhibitor deficiency.
    (ii) Acquired; for example, medication-related.
    b. Primary immune deficiency disorders are seen mainly in 
children. However, recent advances in the treatment of these 
disorders have allowed many affected children to survive well into 
adulthood. Occasionally, these disorders are first diagnosed in 
adolescence or adulthood.
    2. Documentation of immune deficiency disorders. The medical 
evidence must include documentation of the specific type of immune 
deficiency. Documentation may be by laboratory evidence or by other 
generally

[[Page 14604]]

acceptable methods consistent with the prevailing state of medical 
knowledge and clinical practice.
    3. Immune deficiency disorders treated by stem cell 
transplantation.
    a. Evaluation in the first 12 months. If you undergo stem cell 
transplantation for your immune deficiency disorder, we will 
consider you disabled until at least 12 months from the date of the 
transplant.
    b. Evaluation after the 12-month period has elapsed. After the 
12-month period has elapsed, we will consider any residuals of your 
immune deficiency disorder as well as any residual impairment(s) 
resulting from the treatment, such as complications arising from:
    (i) Graft-versus-host (GVH) disease.
    (ii) Immunosuppressant therapy, such as frequent infections.
    (iii) Significant deterioration of other organ systems.
    4. Medication-induced immune suppression. Medication effects can 
result in varying degrees of immune suppression, but most resolve 
when the medication is ceased. However, if you are prescribed 
medication for long-term immune suppression, such as after an organ 
transplant, we will evaluate:
    a. The frequency and severity of infections.
    b. Residuals from the organ transplant itself, after the 12-
month period has elapsed.
    c. Significant deterioration of other organ systems.

F. How do we document and evaluate human immunodeficiency virus 
(HIV) infection?

    Any individual with HIV infection, including one with a 
diagnosis of acquired immune deficiency syndrome (AIDS), may be 
found disabled under 14.08 if his or her impairment meets the 
criteria in that listing or is medically equivalent to the criteria 
in that listing.
    1. Documentation of HIV infection. The medical evidence must 
include documentation of HIV infection. Documentation may be by 
laboratory evidence or by other generally acceptable methods 
consistent with the prevailing state of medical knowledge and 
clinical practice. When you have had laboratory testing for HIV 
infection, we will make every reasonable effort to obtain reports of 
the results of that testing. However, we will not purchase 
laboratory testing to establish whether you have HIV infection.
    a. Definitive documentation of HIV infection. A definitive 
diagnosis of HIV infection is documented by one or more of the 
following laboratory tests:
    (i) HIV antibody tests. HIV antibodies are usually first 
detected by an ELISA screening test performed on serum. Because the 
ELISA can yield false positive results, confirmation is required 
using a more definitive test, such as a Western blot or an 
immunofluorescence assay.
    (ii) Positive ``viral load'' (VL) tests. These tests are 
normally used to quantitate the amount of the virus present but also 
document HIV infection. Such tests include the quantitative plasma 
HIV RNA, quantitative plasma HIV branched DNA, and reverse 
transcriptase-polymerase chain reaction (RT-PCR).
    (iii) HIV DNA detection by polymerase chain reaction (PCR).
    (iv) A specimen that contains HIV antigen (for example, serum 
specimen, lymphocyte culture, or cerebrospinal fluid).
    (v) A positive viral culture for HIV from peripheral blood 
mononuclear cells (PBMC).
    (vi) Other tests that are highly specific for detection of HIV 
and that are consistent with the prevailing state of medical 
knowledge.
    b. Other acceptable documentation of HIV infection. We may also 
document HIV infection without the definitive laboratory evidence 
described in 14.00F1a, provided that such documentation is 
consistent with the prevailing state of medical knowledge and 
clinical practice and is consistent with the other evidence in your 
case record. If no definitive laboratory evidence is available, we 
may document HIV infection by the medical history, clinical and 
laboratory findings, and diagnosis(es) indicated in the medical 
evidence. For example, we will accept a diagnosis of HIV infection 
without definitive laboratory evidence of the HIV infection if you 
have an opportunistic disease that is predictive of a defect in 
cell-mediated immunity (for example, toxoplasmosis of the brain, 
Pneumocystis pneumonia (PCP)), and there is no other known cause of 
diminished resistance to that disease (for example, long-term 
steroid treatment, lymphoma). In such cases, we will make every 
reasonable effort to obtain full details of the history, medical 
findings, and results of testing.
    2. CD4 tests. Individuals who have HIV infection or other 
disorders of the immune system may have tests showing a reduction of 
either the absolute count or the percentage of their T-helper 
lymphocytes (CD4 cells). The extent of immune suppression correlates 
with the level or rate of decline of the CD4 count. Generally, when 
the CD4 count is below 200/mm\3\ (or below 14 percent of the total 
lymphocyte count) the susceptibility to opportunistic infection is 
greatly increased. Although a reduced CD4 count alone does not 
establish a definitive diagnosis of HIV infection, a CD4 count below 
200 does offer supportive evidence when there are clinical findings, 
but not a definitive diagnosis of an opportunistic infection(s). 
However, a reduced CD4 count alone does not document the severity or 
functional consequences of HIV infection.
    3. Documentation of the manifestations of HIV infection. The 
medical evidence must also include documentation of the 
manifestations of HIV infection. Documentation may be by laboratory 
evidence or other generally acceptable methods consistent with the 
prevailing state of medical knowledge and clinical practice.
    a. Definitive documentation of the manifestations of HIV 
infection. The definitive method of diagnosing opportunistic 
diseases or conditions that are manifestations of HIV infection is 
by culture, serologic test, or microscopic examination of biopsied 
tissue or other material (for example, bronchial washings). We will 
make every reasonable effort to obtain specific laboratory evidence 
of an opportunistic disease or other condition whenever this 
information is available. If a histologic or other test has been 
performed, the evidence should include a copy of the appropriate 
report. If we cannot obtain the report, the summary of 
hospitalization or a report from the treating source should include 
details of the findings and results of the diagnostic studies 
(including appropriate medically acceptable imaging studies) or 
microscopic examination of the appropriate tissues or body fluids.
    b. Other acceptable documentation of the manifestations of HIV 
infection. We may also document manifestations of HIV infection 
without the definitive laboratory evidence described in 14.00F3a, 
provided that such documentation is consistent with the prevailing 
state of medical knowledge and clinical practice and is consistent 
with the other evidence in your case record. For example, many 
conditions are now commonly diagnosed based on some or all of the 
following: Medical history, clinical manifestations, laboratory 
findings (including appropriate medically acceptable imaging), and 
treatment responses. In such cases, we will make every reasonable 
effort to obtain full details of the history, medical findings, and 
results of testing. The following are examples of how we may 
document manifestations of HIV infection with other appropriate 
evidence.
    (i) Although a definitive diagnosis of PCP requires identifying 
the organism in bronchial washings, induced sputum, or lung biopsy, 
these tests are frequently bypassed if PCP can be diagnosed 
presumptively. Supportive evidence may include: Fever, dyspnea, 
hypoxia, CD4 count below 200, and no evidence of bacterial 
pneumonia. Also supportive are bilateral lung interstitial 
infiltrates on x-ray, a typical pattern on CAT scan, or a gallium 
scan positive for pulmonary uptake. Response to anti-PCP therapy 
usually requires 5-7 days, and such a response can be supportive of 
the diagnosis.
    (ii) Documentation of Cytomegalovirus (CMV) disease (14.08D) may 
present special problems because definitive diagnosis (except for 
chorioretinitis, which may be diagnosed by an ophthalmologist or 
optometrist on funduscopic examination) requires identification of 
viral inclusion bodies or a positive culture from the affected organ 
and the absence of any other infectious agent likely to be causing 
the disease. A positive serology test does not establish a 
definitive diagnosis of CMV disease, but does offer supportive 
evidence of a presumptive diagnosis of CMV disease. Other clinical 
findings that support a presumptive diagnosis of CMV may include: 
Fever, urinary culture positive for CMV, and CD4 count below 200. A 
clear response to anti-CMV therapy also supports a diagnosis.
    (iii) A definitive diagnosis of toxoplasmosis of the brain is 
based on brain biopsy, but this procedure carries significant risk 
and is not commonly performed. This condition is usually diagnosed 
presumptively based on symptoms or signs of fever, headache, focal 
neurologic deficits, seizures, typical lesions on brain imaging, and 
a positive serology test.
    (iv) Candidiasis of the esophagus (also known as Candida 
esophagitis) may be presumptively diagnosed based on symptoms

[[Page 14605]]

of retrosternal pain on swallowing (odynophagia) and either 
oropharyngeal thrush (white patches or plaques) diagnosed on 
physical examination or by microscopic documentation of Candida 
fungal elements from a noncultured specimen scraped from the oral 
mucosa. Treatment with oral (systemic) antifungal agents usually 
produces improvement after 5 or more days of therapy, and such a 
response can be supportive of the diagnosis.
    4. HIV infection manifestations specific to women.
    a. General. Most women with severe immunosuppression secondary 
to HIV infection exhibit the typical opportunistic infections and 
other conditions, such as PCP, Candida esophagitis, wasting 
syndrome, cryptococcosis, and toxoplasmosis. However, HIV infection 
may have different manifestations in women than in men. Adjudicators 
must carefully scrutinize the medical evidence and be alert to the 
variety of medical conditions specific to, or common in, women with 
HIV infection that may affect their ability to function in the 
workplace.
    b. Additional considerations for evaluating HIV infection in 
women. Many of these manifestations (for example, vulvovaginal 
candidiasis, pelvic inflammatory disease) occur in women with or 
without HIV infection, but can be more severe or resistant to 
treatment, or occur more frequently in a woman whose immune system 
is suppressed. Therefore, when evaluating the claim of a woman with 
HIV infection, it is important to consider gynecologic and other 
problems specific to women, including any associated symptoms (for 
example, pelvic pain), in assessing the severity of the impairment 
and resulting functional limitations. We may evaluate manifestations 
of HIV infection in women under the specific criteria (for example, 
cervical cancer under 14.08E), under an applicable general category 
(for example, pelvic inflammatory disease under 14.08A4) or, in 
appropriate cases, under 14.08K.
    5. Involuntary weight loss. For purposes of 14.08H, an 
involuntary weight loss of at least 10 percent of baseline is always 
considered ``significant.'' Loss of less than 10 percent may or may 
not be significant, depending on the individual's baseline weight 
and body habitus. For example, a 7-pound weight loss in a 100-pound 
woman who is 63 inches tall might be considered significant; but a 
14-pound weight loss in a 200-pound woman who is the same height 
might not be significant. HIV infection that affects the digestive 
system and results in malnutrition can also be evaluated under 5.08.

G. How do we consider the effects of treatment in evaluating your 
autoimmune disorder, immune deficiency disorder, or HIV infection?

    1. General. If your impairment does not otherwise meet the 
requirements of a listing, we will consider your medical treatment 
in terms of its effectiveness in improving the signs, symptoms, and 
laboratory abnormalities of your specific immune system disorder or 
its manifestations, and in terms of any side effects that limit your 
functioning. We will make every reasonable effort to obtain a 
specific description of the treatment you receive (including 
surgery) for your immune system disorder. We consider:
    a. The effects of medications you take.
    b. Adverse side effects (acute and chronic).
    c. The intrusiveness and complexity of your treatment (for 
example, the dosing schedule, need for injections).
    d. The effect of treatment on your mental functioning (for 
example, cognitive changes, mood disturbance).
    e. Variability of your response to treatment (see 14.00G2).
    f. The interactive and cumulative effects of your treatments. 
For example, many individuals with immune system disorders receive 
treatment both for their immune system disorders and for the 
manifestations of the disorders or co-occurring impairments, such as 
treatment for HIV infection and hepatitis C. The interactive and 
cumulative effects of these treatments may be greater than the 
effects of each treatment considered separately.
    g. The duration of your treatment.
    h. Any other aspects of treatment that may interfere with your 
ability to function.
    2. Variability of your response to treatment. Your response to 
treatment and the adverse or beneficial consequences of your 
treatment may vary widely. The effects of your treatment may be 
temporary or long term. For example, some individuals may show an 
initial positive response to a drug or combination of drugs followed 
by a decrease in effectiveness. When we evaluate your response to 
treatment and how your treatment may affect you, we consider such 
factors as disease activity before treatment, requirements for 
changes in therapeutic regimens, the time required for therapeutic 
effectiveness of a particular drug or drugs, the limited number of 
drug combinations that may be available for your impairment(s), and 
the time-limited efficacy of some drugs. For example, an individual 
with HIV infection or another immune deficiency disorder who 
develops pneumonia or tuberculosis may not respond to the same 
antibiotic regimen used in treating individuals without HIV 
infection or another immune deficiency disorder, or may not respond 
to an antibiotic that he or she responded to before. Therefore, we 
must consider the effects of your treatment on an individual basis, 
including the effects of your treatment on your ability to function.
    3. How we evaluate the effects of treatment for autoimmune 
disorders on your ability to function. Some medications may have 
acute or long-term side effects. When we consider the effects of 
corticosteroids or other treatments for autoimmune disorders on your 
ability to function, we consider the factors in 14.00G1 and 14.00G2. 
Long-term corticosteroid treatment can cause ischemic necrosis of 
bone, posterior subcapsular cataract, weight gain, glucose 
intolerance, increased susceptibility to infection, and osteoporosis 
that may result in a loss of function. In addition, medications used 
in the treatment of autoimmune disorders may also have effects on 
mental functioning, including cognition (for example, memory), 
concentration, and mood.
    4. How we evaluate the effects of treatment for immune 
deficiency disorders, excluding HIV infection, on your ability to 
function. When we consider the effects of your treatment for your 
immune deficiency disorder on your ability to function, we consider 
the factors in 14.00G1 and 14.00G2. A frequent need for treatment 
such as intravenous immunoglobulin and gamma interferon therapy can 
be intrusive and interfere with your ability to work. We will also 
consider whether you have chronic side effects from these or other 
medications, including severe fatigue, fever, headaches, high blood 
pressure, joint swelling, muscle aches, nausea, shortness of breath, 
or limitations in mental function including cognition (for example, 
memory), concentration, and mood.
    5. How we evaluate the effects of treatment for HIV infection on 
your ability to function.
    a. General. When we consider the effects of antiretroviral drugs 
(including the effects of highly active antiretroviral therapy 
(HAART)) and the effects of treatments for the manifestations of HIV 
infection on your ability to function, we consider the factors in 
14.00G1 and 14.00G2. Side effects of antiretroviral drugs include, 
but are not limited to: Bone marrow suppression, pancreatitis, 
gastrointestinal intolerance (nausea, vomiting, diarrhea), 
neuropathy, rash, hepatotoxicity, lipodystrophy (fat redistribution, 
such as ``buffalo hump''), glucose intolerance, and lactic acidosis. 
In addition, medications used in the treatment of HIV infection may 
also have effects on mental functioning, including cognition (for 
example, memory), concentration, and mood, and may result in 
malaise, severe fatigue, joint and muscle pain, and insomnia. The 
symptoms of HIV infection and the side effects of medication may be 
indistinguishable from each other. We will consider all of your 
functional limitations, whether they result from your symptoms or 
signs of HIV infection or the side effects of your treatment.
    b. Structured treatment interruptions. A structured treatment 
interruption (STI, also called a ``drug holiday'') is a treatment 
practice during which your treating source advises you to stop 
taking your medications temporarily. An STI in itself does not imply 
that your medical condition has improved; nor does it imply that you 
are noncompliant with your treatment because you are following your 
treating source's advice. Therefore, if you have stopped taking 
medication because your treating source prescribed or recommended an 
STI, we will not find that you are failing to follow treatment or 
draw inferences about the severity of your impairment on this fact 
alone. We will consider why your treating source has prescribed or 
recommended an STI and all the other information in your case record 
when we determine the severity of your impairment.
    6. When there is no record of ongoing treatment. If you have not 
received ongoing treatment or have not had an ongoing relationship 
with the medical community despite the existence of a severe 
impairment(s), we will evaluate the medical severity and duration of 
your immune system disorder on the basis of the current objective 
medical evidence and other evidence in your

[[Page 14606]]

case record, taking into consideration your medical history, 
symptoms, clinical and laboratory findings, and medical source 
opinions. If you have just begun treatment and we cannot determine 
whether you are disabled based on the evidence we have, we may need 
to wait to determine the effect of the treatment on your ability to 
function. The amount of time we need to wait will depend on the 
facts of your case. If you have not received treatment, you may not 
be able to show an impairment that meets the criteria of one of the 
immune system disorders listings, but your immune system disorder 
may medically equal a listing or be disabling based on a 
consideration of your residual functional capacity, age, education, 
and work experience.

H. How do we consider your symptoms, including your pain, severe 
fatigue, and malaise?

    Your symptoms, including pain, severe fatigue, and malaise, may 
be important factors in our determination whether your immune system 
disorder(s) meets or medically equals a listing or in our 
determination whether you are otherwise able to work. In order for 
us to consider your symptoms, you must have medical signs or 
laboratory findings showing the existence of a medically 
determinable impairment(s) that could reasonably be expected to 
produce the symptoms. If you have such an impairment(s), we will 
evaluate the intensity, persistence, and functional effects of your 
symptoms using the rules throughout 14.00 and in our other 
regulations. See Sec. Sec.  404.1528, 404.1529, 416.928, and 
416.929. Additionally, when we assess the credibility of your 
complaints about your symptoms and their functional effects, we will 
not draw any inferences from the fact that you do not receive 
treatment or that you are not following treatment without 
considering all of the relevant evidence in your case record, 
including any explanations you provide that may explain why you are 
not receiving or following treatment.

I. How do we use the functional criteria in these listings?

    1. The following listings in this body system include standards 
for evaluating the functional limitations resulting from immune 
system disorders: 14.02B, for systemic lupus erythematosus; 14.03B, 
for systemic vasculitis; 14.04D, for systemic sclerosis 
(scleroderma); 14.05E, for polymyositis and dermatomyositis; 14.06B, 
for undifferentiated and mixed connective tissue disease; 14.07C, 
for immune deficiency disorders, excluding HIV infection; 14.08K, 
for HIV infection; 14.09D, for inflammatory arthritis; and 14.10B, 
for Sjogren's syndrome.
    2. When we use one of the listings cited in 14.00I1, we will 
consider all relevant information in your case record to determine 
the full impact of your immune system disorder on your ability to 
function on a sustained basis. Important factors we will consider 
when we evaluate your functioning under these listings include, but 
are not limited to: Your symptoms, the frequency and duration of 
manifestations of your immune system disorder, periods of 
exacerbation and remission, and the functional impact of your 
treatment, including the side effects of your medication.
    3. As used in these listings, ``repeated'' means that the 
manifestations occur on an average of three times a year, or once 
every 4 months, each lasting 2 weeks or more; or the manifestations 
do not last for 2 weeks but occur substantially more frequently than 
three times in a year or once every 4 months; or they occur less 
frequently than an average of three times a year or once every 4 
months but last substantially longer than 2 weeks. Your impairment 
will satisfy this criterion regardless of whether you have the same 
kind of manifestation repeatedly, all different manifestations, or 
any other combination of manifestations; for example, two of the 
same kind of manifestation and a different one. You must have the 
required number of manifestations with the frequency and duration 
required in this section. Also, the manifestations must occur within 
the period covered by your claim.
    4. To satisfy the functional criterion in a listing, your immune 
system disorder must result in a ``marked'' level of limitation in 
one of three general areas of functioning: Activities of daily 
living, social functioning, or difficulties in completing tasks due 
to deficiencies in concentration, persistence, or pace. Functional 
limitation may result from the impact of the disease process itself 
on your mental functioning, physical functioning, or both your 
mental and physical functioning. This could result from persistent 
or intermittent symptoms, such as depression, severe fatigue, or 
pain, resulting in a limitation of your ability to do a task, to 
concentrate, to persevere at a task, or to perform the task at an 
acceptable rate of speed. You may also have limitations because of 
your treatment and its side effects (see 14.00G).
    5. When ``marked'' is used as a standard for measuring the 
degree of functional limitation, it means more than moderate but 
less than extreme. We do not define ``marked'' by a specific number 
of different activities of daily living in which your functioning is 
impaired, different behaviors in which your social functioning is 
impaired, or tasks that you are able to complete, but by the nature 
and overall degree of interference with your functioning. You may 
have a marked limitation when several activities or functions are 
impaired, or even when only one is impaired. Also, you need not be 
totally precluded from performing an activity to have a marked 
limitation, as long as the degree of limitation seriously interferes 
with your ability to function independently, appropriately, and 
effectively. The term ``marked'' does not imply that you must be 
confined to bed, hospitalized, or in a nursing home.
    6. Activities of daily living include, but are not limited to, 
such activities as doing household chores, grooming and hygiene, 
using a post office, taking public transportation, or paying bills. 
We will find that you have a ``marked'' limitation of activities of 
daily living if you have a serious limitation in your ability to 
maintain a household or take public transportation because of 
symptoms, such as pain, severe fatigue, anxiety, or difficulty 
concentrating, caused by your immune system disorder (including 
manifestations of the disorder) or its treatment, even if you are 
able to perform some self-care activities.
    7. Social functioning includes the capacity to interact 
independently, appropriately, effectively, and on a sustained basis 
with others. It includes the ability to communicate effectively with 
others. We will find that you have a ``marked'' limitation in 
maintaining social functioning if you have a serious limitation in 
social interaction on a sustained basis because of symptoms, such as 
pain, severe fatigue, anxiety, or difficulty concentrating, or a 
pattern of exacerbation and remission, caused by your immune system 
disorder (including manifestations of the disorder) or its 
treatment, even if you are able to communicate with close friends or 
relatives.
    8. Completing tasks in a timely manner involves the ability to 
sustain concentration, persistence, or pace to permit timely 
completion of tasks commonly found in work settings. We will find 
that you have a ``marked'' limitation in completing tasks if you 
have a serious limitation in your ability to sustain concentration 
or pace adequate to complete work-related tasks because of symptoms, 
such as pain, severe fatigue, anxiety, or difficulty concentrating, 
caused by your immune system disorder (including manifestations of 
the disorder) or its treatment, even if you are able to do some 
routine activities of daily living.

J. How do we evaluate your immune system disorder when it does not 
meet one of these listings?

    1. These listings are only examples of immune system disorders 
that we consider severe enough to prevent you from doing any gainful 
activity. If your impairment(s) does not meet the criteria of any of 
these listings, we must also consider whether you have an 
impairment(s) that satisfies the criteria of a listing in another 
body system.
    2. Individuals with immune system disorders, including HIV 
infection, may manifest signs or symptoms of a mental impairment or 
of another physical impairment. We may evaluate these impairments 
under any affected body system. For example, we will evaluate:
    a. Musculoskeletal involvement, such as surgical reconstruction 
of a joint, under 1.00.
    b. Ocular involvement, such as dry eye, under 2.00.
    c. Respiratory impairments, such as pleuritis, under 3.00.
    d. Cardiovascular impairments, such as cardiomyopathy, under 
4.00.
    e. Digestive impairments, such as hepatitis (including hepatitis 
C) or weight loss as a result of HIV infection that affects the 
digestive system, under 5.00.
    f. Genitourinary impairments, such as nephropathy, under 6.00.
    g. Hematologic abnormalities, such as anemia, granulocytopenia, 
and thrombocytopenia, under 7.00.
    h. Skin impairments, such as persistent fungal and other 
infectious skin eruptions, and photosensitivity, under 8.00.
    i. Neurologic impairments, such as neuropathy or seizures, under 
11.00.

[[Page 14607]]

    j. Mental disorders, such as depression, anxiety, or cognitive 
deficits, under 12.00.
    k. Allergic disorders, such as asthma or atopic dermatitis, 
under 3.00 or 8.00 or under the criteria in another affected body 
system.
    l. Syphilis or neurosyphilis under the criteria for the affected 
body system; for example, 2.00 Special senses and speech, 4.00 
Cardiovascular system, or 11.00 Neurological.
    3. If you have a severe medically determinable impairment(s) 
that does not meet a listing, we will determine whether your 
impairment(s) medically equals a listing. (See Sec. Sec.  404.1526 
and 416.926.) If it does not, you may or may not have the residual 
functional capacity to engage in substantial gainful activity. 
Therefore, we proceed to the fourth, and if necessary, the fifth 
steps of the sequential evaluation process in Sec. Sec.  404.1520 
and 416.920. We use the rules in Sec. Sec.  404.1594, 416.994, and 
416.994a as appropriate, when we decide whether you continue to be 
disabled.
    14.01 Category of Impairments, Immune System Disorders.
    14.02 Systemic lupus erythematosus. As described in 14.00D1. 
With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    B. Repeated manifestations of SLE, with at least two of the 
constitutional symptoms or signs (severe fatigue, fever, malaise, or 
involuntary weight loss) and one of the following at the marked 
level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social functioning.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.
    14.03 Systemic vasculitis. As described in 14.00D2. With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    B. Repeated manifestations of systemic vasculitis, with at least 
two of the constitutional symptoms or signs (severe fatigue, fever, 
malaise, or involuntary weight loss) and one of the following at the 
marked level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social functioning.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.
    14.04 Systemic sclerosis (scleroderma). As described in 14.00D3. 
With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    B. With one of the following:
    1. Toe contractures or fixed deformity of one or both feet, 
resulting in the inability to ambulate effectively as defined in 
14.00C6; or
    2. Finger contractures or fixed deformity in both hands, 
resulting in the inability to perform fine and gross movements 
effectively as defined in 14.00C7; or
    3. Atrophy with irreversible damage in one or both lower 
extremities, resulting in the inability to ambulate effectively as 
defined in 14.00C6; or
    4. Atrophy with irreversible damage in both upper extremities, 
resulting in the inability to perform fine and gross movements 
effectively as defined in 14.00C7.

or

    C. Raynaud's phenomenon, characterized by:
    1. Gangrene involving at least two extremities; or
    2. Ischemia with ulcerations of toes or fingers, resulting in 
the inability to ambulate effectively or to perform fine and gross 
movements effectively as defined in 14.00C6 and 14.00C7;

or

    D. Repeated manifestations of systemic sclerosis (scleroderma), 
with at least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss) and one of the 
following at the marked level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social functioning.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.
    14.05 Polymyositis and dermatomyositis. As described in 14.00D4. 
With:
    A. Proximal limb-girdle (pelvic or shoulder) muscle weakness, 
resulting in inability to ambulate effectively or inability to 
perform fine and gross movements effectively as defined in 14.00C6 
and 14.00C7.

or

    B. Impaired swallowing (dysphagia) with aspiration due to muscle 
weakness.

or

    C. Impaired respiration due to intercostal and diaphragmatic 
muscle weakness.

or

    D. Diffuse calcinosis with limitation of joint mobility or 
intestinal motility.

or

    E. Repeated manifestations of polymyositis or dermatomyositis, 
with at least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss) and one of the 
following at the marked level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social functioning.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.
    14.06 Undifferentiated and mixed connective tissue disease. As 
described in 14.00D5. With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    B. Repeated manifestations of undifferentiated or mixed 
connective tissue disease, with at least two of the constitutional 
symptoms or signs (severe fatigue, fever, malaise, or involuntary 
weight loss) and one of the following at the marked level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social functioning.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.
    14.07 Immune deficiency disorders, excluding HIV infection. As 
described in 14.00E. With:
    A. One or more of the following infections. The infection(s) 
must either be resistant to treatment or require hospitalization or 
intravenous treatment three or more times in a 12-month period.
    1. Sepsis; or
    2. Meningitis; or
    3. Pneumonia; or
    4. Septic arthritis; or
    5. Endocarditis; or
    6. Sinusitis documented by appropriate medically acceptable 
imaging.

or

    B. Stem cell transplantation as described under 14.00E3. 
Consider under a disability until at least 12 months from the date 
of transplantation. Thereafter, evaluate any residual impairment(s) 
under the criteria for the affected body system.

or

    C. Repeated manifestations of an immune deficiency disorder, 
with at least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss) and one of the 
following at the marked level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social function.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.
    14.08 Human immunodeficiency virus (HIV) infection. With 
documentation as described in 14.00F and one of the following:
    A. Bacterial infections:
    1. Mycobacterial infection (for example, caused by M. avium-
intracellulare, M. kansasii, or M. tuberculosis) at a site other 
than the lungs, skin, or cervical or hilar lymph nodes, or pulmonary 
tuberculosis resistant to treatment; or
    2. Nocardiosis; or
    3. Salmonella bacteremia, recurrent non-typhoid; or

[[Page 14608]]

    4. Multiple or recurrent bacterial infections, including pelvic 
inflammatory disease, requiring hospitalization or intravenous 
antibiotic treatment three or more times in a 12-month period. or

    B. Fungal infections:
    1. Aspergillosis; or
    2. Candidiasis involving the esophagus, trachea, bronchi, or 
lungs, or at a site other than the skin, urinary tract, intestinal 
tract, or oral or vulvovaginal mucous membranes; or
    3. Coccidioidomycosis, at a site other than the lungs or lymph 
nodes; or
    4. Cryptococcosis, at a site other than the lungs (for example, 
cryptococcal meningitis); or
    5. Histoplasmosis, at a site other than the lungs or lymph 
nodes; or
    6. Mucormycosis; or
    7. Pneumocystis pneumonia or extrapulmonary Pneumocystis 
infection. or

    C. Protozoan or helminthic infections:
    1. Cryptosporidiosis, isosporiasis, or microsporidiosis, with 
diarrhea lasting for 1 month or longer; or
    2. Strongyloidiasis, extra-intestinal; or
    3. Toxoplasmosis of an organ other than the liver, spleen, or 
lymph nodes. or

    D. Viral infections:
    1. Cytomegalovirus disease (documented as described in 
14.00F3b(ii)) at a site other than the liver, spleen or lymph nodes; 
or
    2. Herpes simplex virus causing:
    a. Mucocutaneous infection (for example, oral, genital, 
perianal) lasting for 1 month or longer; or
    b. Infection at a site other than the skin or mucous membranes 
(for example, bronchitis, pneumonitis, esophagitis, or 
encephalitis); or
    c. Disseminated infection; or
    3. Herpes zoster:
    a. Disseminated; or
    b. With multidermatomal eruptions that are resistant to 
treatment; or
    4. Progressive multifocal leukoencephalopathy.

or

    E. Malignant neoplasms:
    1. Carcinoma of the cervix, invasive, FIGO stage II and beyond; 
or
    2. Kaposi's sarcoma with:
    a. Extensive oral lesions; or
    b. Involvement of the gastrointestinal tract, lungs, or other 
visceral organs; or
    3. Lymphoma (for example, primary lymphoma of the brain, 
Burkitt's lymphoma, immunoblastic sarcoma, other non-Hodgkin's 
lymphoma, Hodgkin's disease); or
    4. Squamous cell carcinoma of the anal canal or anal margin.

or

    F. Conditions of the skin or mucous membranes (other than 
described in B2, D2, or D3, above), with extensive fungating or 
ulcerating lesions not responding to treatment (for example, 
dermatological conditions such as eczema or psoriasis, vulvovaginal 
or other mucosal Candida, condyloma caused by human Papillomavirus, 
genital ulcerative disease).

or

    G. HIV encephalopathy, characterized by cognitive or motor 
dysfunction that limits function and progresses.

or

    H. HIV wasting syndrome, characterized by involuntary weight 
loss of 10 percent or more of baseline (computed based on pounds, 
kilograms, or body mass index (BMI)) or other significant 
involuntary weight loss as described in 14.00F5, and in the absence 
of a concurrent illness that could explain the findings. With 
either:
    1. Chronic diarrhea with two or more loose stools daily lasting 
for 1 month or longer; or
    2. Chronic weakness and documented fever greater than 38[deg]C 
(100.4[deg]F) for the majority of 1 month or longer.

or

    I. Diarrhea, lasting for 1 month or longer, resistant to 
treatment, and requiring intravenous hydration, intravenous 
alimentation, or tube feeding.

or

    J. One or more of the following infections (other than described 
in A-I, above). The infection(s) must either be resistant to 
treatment or require hospitalization or intravenous treatment three 
or more times in a 12-month period.
    1. Sepsis; or
    2. Meningitis; or
    3. Pneumonia; or
    4. Septic arthritis; or
    5. Endocarditis; or
    6. Sinusitis documented by appropriate medically acceptable 
imaging.

or

    K. Repeated (as defined in 14.00I3) manifestations of HIV 
infection, including those listed in 14.08A-J, but without the 
requisite findings for those listings (for example, carcinoma of the 
cervix not meeting the criteria in 14.08E, diarrhea not meeting the 
criteria in 14.08I), or other manifestations (for example, oral 
hairy leukoplakia, myositis, pancreatitis, hepatitis, peripheral 
neuropathy, glucose intolerance, muscle weakness, cognitive or other 
mental limitation) resulting in significant, documented symptoms or 
signs (for example, severe fatigue, fever, malaise, involuntary 
weight loss, pain, night sweats, nausea, vomiting, headaches, or 
insomnia) and one of the following at the marked level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social functioning.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.
    14.09 Inflammatory arthritis. As described in 14.00D6. With:
    A. Persistent inflammation or persistent deformity of:
    1. One or more major peripheral weight-bearing joints resulting 
in the inability to ambulate effectively (as defined in 14.00C6); or
    2. One or more major peripheral joints in each upper extremity 
resulting in the inability to perform fine and gross movements 
effectively (as defined in 14.00C7).

or

    B. Inflammation or deformity in one or more major peripheral 
joints with:
    1. Involvement of two or more organs/body systems with one of 
the organs/body systems involved to at least a moderate level of 
severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    C. Ankylosing spondylitis or other spondyloarthropathies, with:
    1. Ankylosis (fixation) of the dorsolumbar or cervical spine as 
shown by appropriate medically acceptable imaging and measured on 
physical examination at 45[deg] or more of flexion from the vertical 
position (zero degrees); or
    2. Ankylosis (fixation) of the dorsolumbar or cervical spine as 
shown by appropriate medically acceptable imaging and measured on 
physical examination at 30[deg] or more of flexion (but less than 
45[deg]) measured from the vertical position (zero degrees), and 
involvement of two or more organs/body systems with one of the 
organs/body systems involved to at least a moderate level of 
severity.

or

    D. Repeated manifestations of inflammatory arthritis, with at 
least two of the constitutional symptoms or signs (severe fatigue, 
fever, malaise, or involuntary weight loss) and one of the following 
at the marked level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social functioning.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.
    14.10 Sj[ouml]gren's syndrome. As described in 14.00D7. With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    B. Repeated manifestations of Sj[ouml]gren's syndrome, with at 
least two of the constitutional symptoms or signs (severe fatigue, 
fever, malaise, or involuntary weight loss) and one of the following 
at the marked level:
    1. Limitation of activities of daily living.
    2. Limitation in maintaining social functioning.
    3. Limitation in completing tasks in a timely manner due to 
deficiencies in concentration, persistence, or pace.

Part B

* * * * *
    114.00 Immune System Disorders.
* * * * *

101.00 MUSCULOSKELETAL SYSTEM

* * * * *
    B. * * *
    1. * * * The provisions of 101.02 and 101.03 notwithstanding, 
inflammatory arthritis is evaluated under 114.09 (see 114.00D6). * * 
*
* * * * *
    L. * * * When the abnormal curvature of the spine results in 
symptoms related to

[[Page 14609]]

fixation of the dorsolumbar or cervical spine, evaluation of 
equivalence may be made by reference to 114.09C. * * *
* * * * *

108.00 SKIN DISORDERS

* * * * *
    D. * * *
    3. Autoimmune disorders and other immune system disorders (for 
example, systemic lupus erythematosus (SLE), scleroderma, human 
immunodeficiency virus (HIV) infection, and Sj[ouml]gren's syndrome) 
often involve more than one body system. We first evaluate these 
disorders under the immune system disorders listings in 114.00. We 
evaluate SLE under 114.02, scleroderma under 114.04, HIV infection 
under 114.08, and Sj[ouml]gren's syndrome under 114.10.
* * * * *

113.00 MALIGNANT NEOPLASTIC DISEASES

    A. * * * We use the criteria in 114.08E to evaluate carcinoma of 
the cervix, Kaposi's sarcoma, lymphoma, and squamous cell carcinoma 
of the anal canal and anal margin if you also have HIV infection.
* * * * *

114.00 IMMUNE SYSTEM DISORDERS

A. What disorders do we evaluate under the immune system disorders 
listings?

    1. We evaluate immune system disorders that cause dysfunction in 
one or more components of your immune system.
    a. The dysfunction may be due to problems in antibody 
production, impaired cell-mediated immunity, a combined type of 
antibody/cellular deficiency, impaired phagocytosis, or complement 
deficiency.
    b. Immune system disorders may result in recurrent and unusual 
infections, or inflammation and dysfunction of the body's own 
tissues. Immune system disorders can cause a deficit in a single 
organ or body system that results in extreme (that is, very serious) 
loss of function. They can also cause lesser degrees of limitations 
in two or more organs or body systems, and when associated with 
symptoms or signs, such as severe fatigue, fever, malaise, diffuse 
musculoskeletal pain, or involuntary weight loss, can also result in 
extreme limitation. In children, immune system disorders or their 
treatment may also affect growth, development, and the performance 
of age-appropriate activities.
    c. We organize the discussions of immune system disorders in 
three categories: Autoimmune disorders; Immune deficiency disorders, 
excluding human immunodeficiency virus (HIV) infection; and HIV 
infection.
    2. Autoimmune disorders (114.00D). Autoimmune disorders are 
caused by dysfunctional immune responses directed against the body's 
own tissues, resulting in chronic, multisystem impairments that 
differ in clinical manifestations, course, and outcome. They are 
sometimes referred to as rheumatic diseases, connective tissue 
disorders, or collagen vascular disorders. Some of the features of 
autoimmune disorders in children differ from the features of the 
same disorders in adults. The impact of the disorders or their 
treatment on physical, psychological, and developmental growth of 
pre-pubertal children may be considerable, and often differs from 
that of post-pubertal adolescents or adults.
    3. Immune deficiency disorders, excluding HIV infection 
(114.00E). Immune deficiency disorders are characterized by 
recurrent or unusual infections that respond poorly to treatment, 
and are often associated with complications affecting other parts of 
the body. Immune deficiency disorders are classified as either 
primary (congenital) or acquired. Children with immune deficiency 
disorders also have an increased risk of malignancies and of having 
autoimmune disorders.
    4. Human immunodeficiency virus (HIV) infection (114.00F). HIV 
infection may be characterized by increased susceptibility to 
opportunistic infections, cancers, or other conditions, as described 
in 114.08.

B. What information do we need to show that you have an immune 
system disorder?

    Generally, we need your medical history, a report(s) of a 
physical examination, a report(s) of laboratory findings, and in 
some instances, appropriate medically acceptable imaging or tissue 
biopsy reports to show that you have an immune system disorder. 
Therefore, we will make every reasonable effort to obtain your 
medical history, medical findings, and results of laboratory tests. 
We explain the information we need in more detail in the sections 
below.

C. Definitions

    1. Appropriate medically acceptable imaging includes, but is not 
limited to, angiography, x-ray imaging, computerized axial 
tomography (CAT scan) or magnetic resonance imaging (MRI), with or 
without contrast material, myelography, and radionuclear bone scans. 
``Appropriate'' means that the technique used is the proper one to 
support the evaluation and diagnosis of the impairment.
    2. Constitutional symptoms or signs, as used in these listings, 
means severe fatigue, fever, malaise, or involuntary weight loss. 
Severe fatigue means a frequent sense of exhaustion that results in 
significantly reduced physical activity or mental function. Malaise 
means frequent feelings of illness, bodily discomfort, or lack of 
well-being that result in significantly reduced physical activity or 
mental function.
    3. Disseminated means that a condition is spread over a 
considerable area. The type and extent of the spread will depend on 
your specific disease.
    4. Dysfunction means that one or more of the body regulatory 
mechanisms are impaired, causing either an excess or deficiency of 
immunocompetent cells or their products.
    5. Extra-articular means ``other than the joints''; for example, 
an organ(s) such as the heart, lungs, kidneys, or skin.
    6. Inability to ambulate effectively has the same meaning as in 
101.00B2b.
    7. Inability to perform fine and gross movements effectively has 
the same meaning as in 101.00B2c.
    8. Major peripheral joints has the same meaning as in 101.00F.
    9. Persistent means that a sign(s) or symptom(s) has continued 
over time. The precise meaning will depend on the specific immune 
system disorder, the usual course of the disorder, and the other 
circumstances of your clinical course.
    10. Recurrent means that a condition that previously responded 
adequately to an appropriate course of treatment returns after a 
period of remission or regression. The precise meaning, such as the 
extent of response or remission and the time periods involved, will 
depend on the specific disease or condition you have, the body 
system affected, the usual course of the disorder and its treatment, 
and the other facts of your particular case.
    11. Resistant to treatment means that a condition did not 
respond adequately to an appropriate course of treatment. Whether a 
response is adequate or a course of treatment is appropriate will 
depend on the specific disease or condition you have, the body 
system affected, the usual course of the disorder and its treatment, 
and the other facts of your particular case.
    12. Severe means medical severity as used by the medical 
community. The term does not have the same meaning as it does when 
we use it in connection with a finding at the second step of the 
sequential evaluation process in Sec.  416.924.

D. How do we document and evaluate the listed autoimmune disorders?

    1. Systemic lupus erythematosus (114.02).
    a. General. Systemic lupus erythematosus (SLE) is a chronic 
inflammatory disease that can affect any organ or body system. It is 
frequently, but not always, accompanied by constitutional symptoms 
or signs (severe fatigue, fever, malaise, involuntary weight loss). 
Major organ or body system involvement can include: Respiratory 
(pleuritis, pneumonitis), cardiovascular (endocarditis, myocarditis, 
pericarditis, vasculitis), renal (glomerulonephritis), hematologic 
(anemia, leukopenia, thrombocytopenia), skin (photosensitivity), 
neurologic (seizures), mental (anxiety, fluctuating cognition 
(``lupus fog''), mood disorders, organic brain syndrome, psychosis), 
or immune system disorders (inflammatory arthritis). 
Immunologically, there is an array of circulating serum auto-
antibodies and pro- and anti-coagulant proteins that may occur in a 
highly variable pattern.
    b. Documentation of SLE. Generally, but not always, the medical 
evidence will show that your SLE satisfies the criteria in the 
current ``Criteria for the Classification of Systemic Lupus 
Erythematosus'' by the American College of Rheumatology found in the 
most recent edition of the Primer on the Rheumatic Diseases 
published by the Arthritis Foundation.
    2. Systemic vasculitis (114.03).
    a. General.
    (i) Vasculitis is an inflammation of blood vessels. It may occur 
acutely in association with adverse drug reactions, certain chronic 
infections, and occasionally, malignancies. More often, it is 
chronic and the cause is unknown. Symptoms vary depending on which 
blood vessels are involved. Systemic

[[Page 14610]]

vasculitis may also be associated with other autoimmune disorders; 
for example, SLE or dermatomyositis.
    (ii) Children can develop the vasculitis of Kawasaki disease, of 
which the most serious manifestation is formation of coronary artery 
aneurysms and related complications. We evaluate heart problems 
related to Kawasaki disease under the criteria in the cardiovascular 
listings (104.00). Children can also develop the vasculitis of 
anaphylactoid purpura (Henoch-Schoenlein purpura), which may cause 
intestinal and renal disorders. We evaluate intestinal and renal 
disorders related to vasculitis of anaphylactoid purpura under the 
criteria in the digestive (105.00) or genitourinary (106.00) 
listings. Other clinical patterns include, but are not limited to, 
polyarteritis nodosa, Takayasu's arteritis (aortic arch arteritis), 
and Wegener's granulomatosis.
    b. Documentation of systemic vasculitis. Angiography or tissue 
biopsy confirms a diagnosis of systemic vasculitis when the disease 
is suspected clinically. When you have had angiography or tissue 
biopsy for systemic vasculitis, we will make every reasonable effort 
to obtain reports of the results of that procedure. However, we will 
not purchase angiography or tissue biopsy.
    3. Systemic sclerosis (scleroderma) (114.04).
    a. General. Systemic sclerosis (scleroderma) constitutes a 
spectrum of disease in which thickening of the skin is the clinical 
hallmark. Raynaud's phenomenon, often medically severe and 
progressive, is present frequently and may be the peripheral 
manifestation of a vasospastic abnormality in the heart, lungs, and 
kidneys. The CREST syndrome (calcinosis, Raynaud's phenomenon, 
esophageal dysmotility, sclerodactyly, and telangiectasia) is a 
variant that may slowly progress over years to the generalized 
process, systemic sclerosis.
    b. Diffuse cutaneous systemic sclerosis. In diffuse cutaneous 
systemic sclerosis (also known as diffuse scleroderma), major organ 
or systemic involvement can include the gastrointestinal tract, 
lungs, heart, kidneys, and muscle in addition to skin or blood 
vessels. Although arthritis can occur, joint dysfunction results 
primarily from soft tissue/cutaneous thickening, fibrosis, and 
contractures.
    c. Localized scleroderma (linear scleroderma and morphea).
    (i) Localized scleroderma (linear scleroderma and morphea) is 
more common in children than systemic scleroderma. To assess the 
severity of the impairment, we need a description of the extent of 
involvement of linear scleroderma and the location of the lesions. 
For example, linear scleroderma involving the arm but not crossing 
any joints is not as functionally limiting as sclerodactyly 
(scleroderma localized to the fingers). Linear scleroderma of a 
lower extremity involving skin thickening and atrophy of underlying 
muscle or bone can result in contractures and leg length 
discrepancy. In such cases, we may evaluate your impairment under 
the musculoskeletal listings (101.00).
    (ii) When there is isolated morphea of the face causing facial 
disfigurement from unilateral hypoplasia of the mandible, maxilla, 
zygoma, or orbit, adjudication may be more appropriate under the 
criteria in the affected body system, such as special senses and 
speech (102.00) or mental disorders (112.00).
    (iii) Chronic variants of these syndromes include disseminated 
morphea, Shulman's disease (diffuse fasciitis with eosinophilia), 
and eosinophilia-myalgia syndrome (often associated with toxins such 
as toxic oil or contaminated tryptophan), all of which can impose 
medically severe musculoskeletal dysfunction and may also lead to 
restrictive pulmonary disease. We evaluate these variants of the 
disease under the criteria in the musculoskeletal listings (101.00) 
or respiratory system listings (103.00).
    d. Documentation of systemic sclerosis (scleroderma). 
Documentation involves differentiating the clinical features of 
systemic sclerosis (scleroderma) from other autoimmune disorders. 
However, there may be an overlap.
    4. Polymyositis and dermatomyositis (114.05).
    a. General.
    (i) Polymyositis and dermatomyositis are related disorders that 
are characterized by an inflammatory process in striated muscle, 
occurring alone or in association with other autoimmune disorders. 
The most common manifestations are symmetric weakness, and less 
frequently, pain and tenderness of the proximal limb-girdle 
(shoulder or pelvic) musculature. There may also be involvement of 
the cervical, cricopharyngeal, esophageal, intercostal, and 
diaphragmatic muscles.
    (ii) Polymyositis occurs rarely in children; the more common 
presentation in children is dermatomyositis with symmetric proximal 
muscle weakness and characteristic skin findings. The clinical 
course of dermatomyositis can be more severe when it is accompanied 
by systemic vasculitis rather than just localized to striated 
muscle. Late in the disease, some children with dermatomyositis 
develop calcinosis of the skin and subcutaneous tissues, muscles, 
and joints. We evaluate the involvement of other organs/body systems 
under the criteria for the listings in the affected body system.
    b. Documentation of polymyositis and dermatomyositis. Generally, 
but not always, polymyositis is associated with elevated serum 
muscle enzymes (creatine phosphokinase (CPK), aminotransferases, and 
aldolase), and characteristic abnormalities on electromyography and 
muscle biopsy. In children, the diagnosis of dermatomyositis is 
supported largely by medical history, findings on physical 
examination that include the characteristic skin findings, and 
elevated serum muscle enzymes. Muscle inflammation or vasculitis 
depicted on MRI is additional evidence supporting the diagnosis of 
childhood dermatomyositis. When you have had electromyography, 
muscle biopsy, or MRI for polymyositis or dermatomyositis, we will 
make every reasonable effort to obtain reports of the results of 
that procedure. However, we will not purchase electromyography, 
muscle biopsy, or MRI.
    c. Additional information about how we evaluate polymyositis and 
dermatomyositis under the listings.
    (i) In newborn and younger infants (birth to attainment of age 
1), we consider muscle weakness that affects motor skills, such as 
head control, reaching, grasping, taking solids, or self-feeding, 
under 114.05A. In older infants and toddlers (age 1 to attainment of 
age 3), we also consider muscle weakness affecting your ability to 
roll over, sit, crawl, or walk under 114.05A.
    (ii) If you are of preschool age through adolescence (age 3 to 
attainment of age 18), weakness of your pelvic girdle muscles that 
results in your inability to rise independently from a squatting or 
sitting position or to climb stairs may be an indication that you 
are unable to ambulate effectively. Weakness of your shoulder girdle 
muscles may result in your inability to perform lifting, carrying, 
and reaching overhead, and also may seriously affect your ability to 
perform activities requiring fine movements. We evaluate these 
limitations under 114.05A.
    5. Undifferentiated and mixed connective tissue disease 
(114.06).
    a. General. This listing includes syndromes with clinical and 
immunologic features of several autoimmune disorders, but which do 
not satisfy the criteria for any of the specific disorders 
described. For example, you may have clinical features of SLE and 
systemic vasculitis, and the serologic (blood test) findings of 
rheumatoid arthritis. The most common pattern of undifferentiated 
autoimmune disorders in children is mixed connective tissue disease 
(MCTD).
    b. Documentation of undifferentiated and mixed connective tissue 
disease. Undifferentiated connective tissue disease is diagnosed 
when clinical features and serologic (blood test) findings, such as 
rheumatoid factor or antinuclear antibody (consistent with an 
autoimmune disorder) are present but do not satisfy the criteria for 
a specific disease. Children with MCTD have laboratory findings of 
extremely high antibody titers to extractable nuclear antigen (ENA) 
or ribonucleoprotein (RNP) without high titers of anti-dsDNA or 
anti-SM antibodies. There are often clinical findings suggestive of 
SLE or childhood dermatomyositis. Many children later develop 
features of scleroderma.
    6. Inflammatory arthritis (114.09).
    a. General. The spectrum of inflammatory arthritis includes a 
vast array of disorders that differ in cause, course, and outcome. 
Clinically, inflammation of major peripheral joints may be the 
dominant manifestation causing difficulties with ambulation or fine 
and gross movements; there may be joint pain, swelling, and 
tenderness. The arthritis may affect other joints, or cause less 
limitation in ambulation or the performance of fine and gross 
movements. However, in combination with extra-articular features, 
including constitutional symptoms or signs (severe fatigue, fever, 
malaise, involuntary weight loss), inflammatory arthritis may result 
in an extreme limitation. You may also have impaired growth as a 
result of the inflammatory arthritis because of its effects on the 
immature skeleton, open epiphyses, and young cartilage and bone. We 
evaluate any associated growth impairment under the criteria in 
100.00.
    b. Inflammatory arthritis involving the axial spine 
(spondyloarthropathy). In

[[Page 14611]]

children, inflammatory arthritis involving the axial spine may be 
associated with disorders such as:
    (i) Reactive arthropathies;
    (ii) Juvenile ankylosing spondylitis;
    (iii) Psoriatic arthritis;
    (iv) SEA syndrome (seronegative enthesopathy arthropathy 
syndrome);
    (v) Beh[ccedil]et's disease; and
    (vi) Inflammatory bowel disease.
    c. Inflammatory arthritis involving the peripheral joints. In 
children, inflammatory arthritis involving peripheral joints may be 
associated with disorders such as:
    (i) Juvenile rheumatoid arthritis;
    (ii) Sjogren's syndrome;
    (iii) Psoriatic arthritis;
    (iv) Crystal deposition disorders (gout and pseudogout);
    (v) Lyme disease; and
    (vi) Inflammatory bowel disease.
    d. Documentation of inflammatory arthritis. Generally, but not 
always, the diagnosis of inflammatory arthritis is based on the 
clinical features and serologic findings described in the most 
recent edition of the Primer on the Rheumatic Diseases published by 
the Arthritis Foundation.
    e. How we evaluate inflammatory arthritis under the listings.
    (i) Listing-level severity in 114.09A and 114.09C1 is shown by 
an impairment that results in an ``extreme'' (very serious) 
limitation. In 114.09A, the criterion is satisfied with persistent 
inflammation or deformity in one major peripheral weight-bearing 
joint resulting in the inability to ambulate effectively (as defined 
in 114.00C6) or one major peripheral joint in each upper extremity 
resulting in the inability to perform fine and gross movements 
effectively (as defined in 114.00C7). In 114.09C1, if you have the 
required ankylosis (fixation) of your cervical or dorsolumbar spine, 
we will find that you have an extreme limitation in your ability to 
see in front of you, above you, and to the side. Therefore, 
inability to ambulate effectively is implicit in 114.09C1, even 
though you might not require bilateral upper limb assistance.
    (ii) Listing-level severity is shown in 114.09B, 114.09C2, and 
114.09D by inflammatory arthritis that involves various combinations 
of complications of one or more major peripheral joints or involves 
other joints, such as inflammation or deformity, extra-articular 
features, repeated manifestations, and constitutional symptoms and 
signs. Extra-articular impairments may also meet listings in other 
body systems.
    (iii) Extra-articular features of inflammatory arthritis may 
involve any body system; for example: Musculoskeletal (heel 
enthesopathy), ophthalmologic (iridocyclitis, keratoconjunctivitis 
sicca, uveitis), pulmonary (pleuritis, pulmonary fibrosis or 
nodules, restrictive lung disease), cardiovascular (aortic valve 
insufficiency, arrhythmias, coronary arteritis, myocarditis, 
pericarditis, Raynaud's phenomenon, systemic vasculitis), renal 
(amyloidosis of the kidney), hematologic (chronic anemia, 
thrombocytopenia), neurologic (peripheral neuropathy, radiculopathy, 
spinal cord or cauda equina compression with sensory and motor 
loss), mental (cognitive dysfunction, poor memory), and immune 
system (Felty's syndrome (hypersplenism with compromised immune 
competence)).
    (iv) If both inflammation and chronic deformities are present, 
we evaluate your impairment under the criteria of any appropriate 
listing.
    7. Sj[ouml]gren's syndrome (114.10).
    a. General.
    (i) Sj[ouml]gren's syndrome is an immune-mediated disorder of 
the exocrine glands. Involvement of the lacrimal and salivary glands 
is the hallmark feature, resulting in symptoms of dry eyes and dry 
mouth, and possible complications, such as corneal damage, 
blepharitis (eyelid inflammation), dysphagia (difficulty in 
swallowing), dental caries, and the inability to speak for extended 
periods of time. Involvement of the exocrine glands of the upper 
airways may result in persistent dry cough.
    (ii) Many other organ systems may be involved, including 
musculoskeletal (arthritis, myositis), respiratory (interstitial 
fibrosis), gastrointestinal (dysmotility, dysphagia, involuntary 
weight loss), genitourinary (interstitial cystitis, renal tubular 
acidosis), skin (purpura, vasculitis,), neurologic (central nervous 
system disorders, cranial and peripheral neuropathies), mental 
(cognitive dysfunction, poor memory), and neoplastic (lymphoma). 
Severe fatigue and malaise are frequently reported. Sj[ouml]gren's 
syndrome may be associated with other autoimmune disorders (for 
example, rheumatoid arthritis or SLE); usually the clinical features 
of the associated disorder predominate.
    b. Documentation of Sj[ouml]gren's syndrome. If you have 
Sj[ouml]gren's syndrome, the medical evidence will generally, but 
not always, show that your disease satisfies the criteria in the 
current ``Criteria for the Classification of Sj[ouml]gren's 
Syndrome'' by the American College of Rheumatology found in the most 
recent edition of the Primer on the Rheumatic Diseases published by 
the Arthritis Foundation.

E. How do we document and evaluate immune deficiency disorders, 
excluding HIV infection?

    1. General.
    a. Immune deficiency disorders can be classified as:
    (i) Primary (congenital); for example, X-linked 
agammaglobulinemia, thymic hypoplasia (DiGeorge syndrome), severe 
combined immunodeficiency (SCID), chronic granulomatous disease 
(CGD), C1 esterase inhibitor deficiency.
    (ii) Acquired; for example, medication-related.
    b. Primary immune deficiency disorders are seen mainly in 
children. However, recent advances in the treatment of these 
disorders have allowed many affected children to survive well into 
adulthood. Occasionally, these disorders are first diagnosed in 
adolescence or adulthood.
    2. Documentation of immune deficiency disorders. The medical 
evidence must include documentation of the specific type of immune 
deficiency. Documentation may be by laboratory evidence or by other 
generally acceptable methods consistent with the prevailing state of 
medical knowledge and clinical practice.
    3. Immune deficiency disorders treated by stem cell 
transplantation.
    a. Evaluation in the first 12 months. If you undergo stem cell 
transplantation for your immune deficiency disorder, we will 
consider you disabled until at least 12 months from the date of the 
transplant.
     b. Evaluation after the 12-month period has elapsed. After the 
12-month period has elapsed, we will consider any residuals of your 
immune deficiency disorder as well as any residual impairment(s) 
resulting from the treatment, such as complications arising from:
    (i) Graft-versus-host (GVH) disease.
    (ii) Immunosuppressant therapy, such as frequent infections.
    (iii) Significant deterioration of other organ systems.
    4. Medication-induced immune suppression. Medication effects can 
result in varying degrees of immune suppression, but most resolve 
when the medication is ceased. However, if you are prescribed 
medication for long-term immune suppression, such as after an organ 
transplant, we will evaluate:
    a. The frequency and severity of infections.
    b. Residuals from the organ transplant itself, after the 12-
month period has elapsed.
    c. Significant deterioration of other organ systems.
    F. How do we document and evaluate human immunodeficiency virus 
(HIV) infection? Any child with HIV infection, including one with a 
diagnosis of acquired immune deficiency syndrome (AIDS), may be 
found disabled under 114.08 if his or her impairment meets the 
criteria in that listing or is medically equivalent to the criteria 
in that listing.
    1. Documentation of HIV infection. The medical evidence must 
include documentation of HIV infection. Documentation may be by 
laboratory evidence or by other generally acceptable methods 
consistent with the prevailing state of medical knowledge and 
clinical practice. When you have had laboratory testing for HIV 
infection, we will make every reasonable effort to obtain reports of 
the results of that testing. However, we will not purchase 
laboratory testing to establish whether you have HIV infection.
    a. Definitive documentation of HIV infection. A definitive 
diagnosis of HIV infection is documented by one or more of the 
following laboratory tests:
    (i) HIV antibody tests. HIV antibodies are usually first 
detected by an ELISA screening test performed on serum. Because the 
ELISA can yield false positive results, confirmation is required 
using a more definitive test, such as a Western blot or an 
immunofluorescence assay. Positive results on these tests are 
considered to be diagnostic of HIV infection in a child age 18 
months or older. (See b. below for information about HIV antibody 
testing in children younger than 18 months of age.)
    (ii) Positive ``viral load'' (VL) tests. These tests are 
normally used to quantitate the amount of the virus present but also 
document HIV infection. Such tests include the quantitative plasma 
HIV RNA,

[[Page 14612]]

quantitative plasma HIV branched DNA, and reverse transcriptase-
polymerase chain reaction (RT-PCR).
    (iii) HIV DNA detection by polymerase chain reaction (PCR).
    (iv) A specimen that contains HIV antigen (for example, serum 
specimen, lymphocyte culture, or cerebrospinal fluid) in a child age 
1 month or older.
    (v) A positive viral culture for HIV from peripheral blood 
mononuclear cells (PBMC).
    (vi) An immunoglobulin A (IgA) serological assay that is 
specific for HIV.
    (vii) Other tests that are highly specific for detection of HIV 
and that are consistent with the prevailing state of medical 
knowledge.
    b. Definitive documentation of HIV infection in children from 
birth to the attainment of 18 months. For children from birth to the 
attainment of 18 months of age, and who have tested positive for HIV 
antibodies, HIV infection is documented by:
    (i) One or more of the tests listed in F1a(ii)-F1a(vii).
    (ii) For newborn and younger infants (birth to attainment of age 
1), a CD4 (T4) count of 1500/mm\3\ or less, or a CD4 count less than 
or equal to 20 percent of total lymphocytes.
    (iii) For older infants and toddlers from 12 to 18 months of 
age, a CD4 (T4) count of 750/mm\3\ or less, or a CD4 count less than 
or equal to 20 percent of total lymphocytes.
    (iv) An abnormal CD4/CD8 ratio.
    (v) A severely diminished immunoglobulin G (IgG) level (< 4 g/l 
or 400 mg/dl), or significantly greater than normal range for age.
    c. Other acceptable documentation of HIV infection. We may also 
document HIV infection without the definitive laboratory evidence 
described in 114.00F1a, provided that such documentation is 
consistent with the prevailing state of medical knowledge and 
clinical practice and is consistent with the other evidence in your 
case record. If no definitive laboratory evidence is available, we 
may document HIV infection by the medical history, clinical and 
laboratory findings, and diagnosis(es) indicated in the medical 
evidence. For example, we will accept a diagnosis of HIV infection 
without definitive laboratory evidence of the HIV infection if you 
have an opportunistic disease that is predictive of a defect in 
cell-mediated immunity (for example, Pneumocystis pneumonia (PCP)), 
and there is no other known cause of diminished resistance to that 
disease (for example, long-term steroid treatment, lymphoma). In 
such cases, we will make every reasonable effort to obtain full 
details of the history, medical findings, and results of testing.
    2. CD4 tests. Children who have HIV infection or other disorders 
of the immune system may have tests showing a reduction of either 
the absolute count or the percentage of their T-helper lymphocytes 
(CD4 cells). The extent of immune suppression correlates with the 
level or rate of decline of the CD4 count (relative to the age of 
the young child). By age 6, children have CD4 counts comparable to 
those levels found in adults. Generally, in these children when the 
CD4 count is below 200/mm\3\ (or below 14 percent of the total 
lymphocyte count) the susceptibility to opportunistic infection is 
greatly increased. Although a reduced CD4 count alone does not 
establish a definitive diagnosis of HIV infection, a CD4 count below 
200 does offer supportive evidence when there are clinical findings, 
but not a definitive diagnosis of an opportunistic infection(s). 
However, a reduced CD4 count alone does not document the severity or 
functional consequences of HIV infection.
    3. Documentation of the manifestations of HIV infection. The 
medical evidence must also include documentation of the 
manifestations of HIV infection. Documentation may be by laboratory 
evidence or other generally acceptable methods consistent with the 
prevailing state of medical knowledge and clinical practice.
    a. Definitive documentation of the manifestations of HIV 
infection. The definitive method of diagnosing opportunistic 
diseases or conditions that are manifestations of HIV infection is 
by culture, serologic test, or microscopic examination of biopsied 
tissue or other material (for example, bronchial washings). We will 
make every reasonable effort to obtain specific laboratory evidence 
of an opportunistic disease or other condition whenever this 
information is available. If a histologic or other test has been 
performed, the evidence should include a copy of the appropriate 
report. If we cannot obtain the report, the summary of 
hospitalization or a report from the treating source should include 
details of the findings and results of the diagnostic studies 
(including appropriate medically acceptable imaging studies) or 
microscopic examination of the appropriate tissues or body fluids.
    b. Other acceptable documentation of the manifestations of HIV 
infection. We may also document manifestations of HIV infection 
without the definitive laboratory evidence described in 114.00F3a, 
provided that such documentation is consistent with the prevailing 
state of medical knowledge and clinical practice and is consistent 
with the other evidence in your case record. For example, many 
conditions are now commonly diagnosed based on some or all of the 
following: Medical history, clinical manifestations, laboratory 
findings (including appropriate medically acceptable imaging), and 
treatment responses. In such cases, we will make every reasonable 
effort to obtain full details of the history, medical findings, and 
results of testing. The following are examples of how we may 
document manifestations of HIV infection with other appropriate 
evidence.
    (i) Although a definitive diagnosis of PCP requires identifying 
the organism in bronchial washings, induced sputum, or lung biopsy, 
these tests are frequently bypassed if PCP can be diagnosed 
presumptively. Supportive evidence may include: Fever, dyspnea, 
hypoxia, CD4 count below 200 in children 6 years of age or older, 
and no evidence of bacterial pneumonia. Also supportive are 
bilateral lung interstitial infiltrates on x-ray, a typical pattern 
on CAT scan, or a gallium scan positive for pulmonary uptake. 
Response to anti-PCP therapy usually requires 5-7 days, and such a 
response can be supportive of the diagnosis.
    (ii) Documentation of Cytomegalovirus (CMV) disease (114.08D) 
may present special problems because definitive diagnosis (except 
for chorioretinitis, which may be diagnosed by an ophthalmologist or 
optometrist on funduscopic examination) requires identification of 
viral inclusion bodies or a positive culture from the affected organ 
and the absence of any other infectious agent likely to be causing 
the disease. A positive serology test does not establish a 
definitive diagnosis of CMV disease, but does offer supportive 
evidence of a presumptive diagnosis of CMV disease. Other clinical 
findings that support a presumptive diagnosis of CMV may include: 
Fever, urinary culture positive for CMV, and CD4 count below 200 in 
children 6 years of age or older. A clear response to anti-CMV 
therapy also supports a diagnosis.
    (iii) A definitive diagnosis of toxoplasmosis of the brain is 
based on brain biopsy, but this procedure carries significant risk 
and is not commonly performed. This condition is usually diagnosed 
presumptively based on symptoms or signs of fever, headache, focal 
neurologic deficits, seizures, typical lesions on brain imaging, and 
a positive serology test.
    (iv) Candidiasis of the esophagus (also known as Candida 
esophagitis) may be presumptively diagnosed based on symptoms of 
retrosternal pain on swallowing (odynophagia) and either 
oropharyngeal thrush (white patches or plaques) diagnosed on 
physical examination or by microscopic documentation of Candida 
fungal elements from a noncultured specimen scraped from the oral 
mucosa. Treatment with oral (systemic) antifungal agents usually 
produces improvement after 5 or more days of therapy, and such a 
response can be supportive of the diagnosis.
    4. HIV infection manifestations specific to children.
    a. General. The clinical manifestation and course of disease in 
children who become infected with HIV perinatally or in the first 12 
years of life may differ from that in adolescents (age 12 to 
attainment of age 18) and adults. Newborn and younger infants (birth 
to attainment of age 1) and older infants and toddlers (age 1 to 
attainment of age 3) may present with failure to thrive or PCP; 
preschool children (age 3 to attainment of age 6) and primary school 
children (age 6 to attainment of age 12) may present with recurrent 
infections, neurological problems, or developmental abnormalities. 
Adolescents may also exhibit neurological abnormalities, such as HIV 
encephalopathy, or have growth problems. HIV infection that affects 
the digestive system and results in malnutrition also may be 
evaluated under 105.08.
    b. Neurologic abnormalities. The methods of identifying and 
evaluating neurologic abnormalities may vary depending on a child's 
age. For example, in an infant, impaired brain growth can be 
documented by a decrease in the growth rate of the head. In an older 
child, impaired brain growth may be documented by brain atrophy on a 
CAT scan or MRI. Neurologic abnormalities in infants and young 
children may present as serious developmental delays or in the loss 
of previously acquired developmental

[[Page 14613]]

milestones. In school-age children and adolescents, this type of 
neurologic abnormality generally presents as the loss of previously 
acquired intellectual abilities. This may be evidenced in a child by 
a decrease in intelligence quotient (IQ) scores, by forgetting 
information previously learned, by inability to learn new 
information, or by a sudden onset of a new learning disability.
    c. Bacterial infections. Children with HIV infection may 
contract any of a broad range of bacterial infections. Certain major 
infections caused by pyogenic bacteria (for example, some 
pneumonias) can be severely limiting, especially in pre-adolescent 
children. We evaluate these major bacterial infections under 
114.08A4. Although 114.08A4 applies only to children under 13 years 
of age, children age 13 and older may have an impairment that 
medically equals this listing if the circumstances of the case 
warrant; for example, if there is delayed puberty. We will evaluate 
pelvic inflammatory disease in older girls under 114.08A5.

G. How do we consider the effects of treatment in evaluating your 
autoimmune disorder, immune deficiency disorder, or HIV infection?

    1. General. If your impairment does not otherwise meet the 
requirements of a listing, we will consider your medical treatment 
in terms of its effectiveness in improving the signs, symptoms, and 
laboratory abnormalities of your specific immune system disorder or 
its manifestations, and in terms of any side effects that limit your 
functioning. We will make every reasonable effort to obtain a 
specific description of the treatment you receive (including 
surgery) for your immune system disorder. We consider:
    a. The effects of medications you take.
    b. Adverse side effects (acute and chronic).
    c. The intrusiveness and complexity of your treatment (for 
example, the dosing schedule, need for injections).
    d. The effect of treatment on your mental functioning (for 
example, cognitive changes, mood disturbance).
    e. Variability of your response to treatment (see 114.00G2).
    f. The interactive and cumulative effects of your treatments. 
For example, many children with immune system disorders receive 
treatment both for their immune system disorders and for the 
manifestations of the disorders or co-occurring impairments, such as 
treatment for HIV infection and hepatitis C. The interactive and 
cumulative effects of these treatments may be greater than the 
effects of each treatment considered separately.
    g. The duration of your treatment.
    h. Any other aspects of treatment that may interfere with your 
ability to function.
    2. Variability of your response to treatment. Your response to 
treatment and the adverse or beneficial consequences of your 
treatment may vary widely. The effects of your treatment may be 
temporary or long term. For example, some children may show an 
initial positive response to a drug or combination of drugs followed 
by a decrease in effectiveness. When we evaluate your response to 
treatment and how your treatment may affect you, we consider such 
factors as disease activity before treatment, requirements for 
changes in therapeutic regimens, the time required for therapeutic 
effectiveness of a particular drug or drugs, the limited number of 
drug combinations that may be available for your impairment(s), and 
the time-limited efficacy of some drugs. For example, a child with 
HIV infection or another immune deficiency disorder who develops 
otitis media may not respond to the same antibiotic regimen used in 
treating children without HIV infection or another immune deficiency 
disorder, or may not respond to an antibiotic that he or she 
responded to before. Therefore, we must consider the effects of your 
treatment on an individual basis, including the effects of your 
treatment on your ability to function.
    3. How we evaluate the effects of treatment for autoimmune 
disorders on your ability to function. Some medications may have 
acute or long-term side effects. When we consider the effects of 
corticosteroids or other treatments for autoimmune disorders on your 
ability to function, we consider the factors in 114.00G1 and 
114.00G2. Long-term corticosteroid treatment can cause ischemic 
necrosis of bone, posterior subcapsular cataract, impaired growth, 
weight gain, glucose intolerance, increased susceptibility to 
infection, and osteopenia that may result in a loss of function. In 
addition, medications used in the treatment of autoimmune disorders 
may also have effects on mental functioning, including cognition 
(for example, memory), concentration, and mood.
    4. How we evaluate the effects of treatment for immune 
deficiency disorders, excluding HIV infection, on your ability to 
function. When we consider the effects of your treatment for your 
immune deficiency disorder on your ability to function, we consider 
the factors in 114.00G1 and 114.00G2. A frequent need for treatment 
such as intravenous immunoglobulin and gamma interferon therapy can 
be intrusive and interfere with your ability to function. We will 
also consider whether you have chronic side effects from these or 
other medications, including severe fatigue, fever, headaches, high 
blood pressure, joint swelling, muscle aches, nausea, shortness of 
breath, or limitations in mental function including cognition (for 
example, memory) concentration, and mood.
    5. How we evaluate the effects of treatment for HIV infection on 
your ability to function.
    a. General. When we consider the effects of antiretroviral drugs 
(including the effects of highly active antiretroviral therapy 
(HAART)) and the effects of treatments for the manifestations of HIV 
infection on your ability to function, we consider the factors in 
114.00G1 and 114.00G2. Side effects of antiretroviral drugs include, 
but are not limited to: Bone marrow suppression, pancreatitis, 
gastrointestinal intolerance (nausea, vomiting, diarrhea), 
neuropathy, rash, hepatotoxicity, lipodystrophy (fat redistribution, 
such as ``buffalo hump''), glucose intolerance, and lactic acidosis. 
In addition, medications used in the treatment of HIV infection may 
also have effects on mental functioning, including cognition (for 
example, memory), concentration, and mood, and may result in 
malaise, severe fatigue, joint and muscle pain, and insomnia. The 
symptoms of HIV infection and the side effects of medication may be 
indistinguishable from each other. We will consider all of your 
functional limitations, whether they result from your symptoms or 
signs of HIV infection or the side effects of your treatment.
    b. Structured treatment interruptions. A structured treatment 
interruption (STI, also called a ``drug holiday'') is a treatment 
practice during which your treating source advises you to stop 
taking your medications temporarily. An STI in itself does not imply 
that your medical condition has improved; nor does it imply that you 
are noncompliant with your treatment because you are following your 
treating source's advice. Therefore, if you have stopped taking 
medication because your treating source prescribed or recommended an 
STI, we will not find that you are failing to follow treatment or 
draw inferences about the severity of your impairment on this fact 
alone. We will consider why your treating source has prescribed or 
recommended an STI and all the other information in your case record 
when we determine the severity of your impairment.
    6. When there is no record of ongoing treatment. If you have not 
received ongoing treatment or have not had an ongoing relationship 
with the medical community despite the existence of a severe 
impairment(s), we will evaluate the medical severity and duration of 
your immune system disorder on the basis of the current objective 
medical evidence and other evidence in your case record, taking into 
consideration your medical history, symptoms, clinical and 
laboratory findings, and medical source opinions. If you have just 
begun treatment and we cannot determine whether you are disabled 
based on the evidence we have, we may need to wait to determine the 
effect of the treatment on your ability to develop and function in 
an age-appropriate manner. The amount of time we need to wait will 
depend on the facts of your case. If you have not received 
treatment, you may not be able to show an impairment that meets the 
criteria of one of the immune system disorders listings, but your 
immune system disorder may medically equal a listing or functionally 
equal the listings.

H. How do we consider your symptoms, including your pain, severe 
fatigue, and malaise?

    Your symptoms, including pain, severe fatigue, and malaise, may 
be important factors in our determination whether your immune system 
disorder(s) meets or medically equals a listing or in our 
determination whether you otherwise have marked and severe 
functional limitations. In order for us to consider your symptoms, 
you must have medical signs or laboratory findings showing the 
existence of a medically determinable impairment(s) that could 
reasonably be expected to produce the symptoms. If you have such an 
impairment(s), we will evaluate the intensity, persistence, and 
functional effects of your symptoms using the rules throughout 
114.00 and in our other regulations. See Sec. Sec.  416.928,

[[Page 14614]]

and 416.929. Additionally, when we assess the credibility of your 
complaints about your symptoms and their functional effects, we will 
not draw any inferences from the fact that you do not receive 
treatment or that you are not following treatment without 
considering all of the relevant evidence in your case record, 
including any explanations you provide that may explain why you are 
not receiving or following treatment.

I. How do we use the functional criteria in these listings?

    1. The following listings in this body system include standards 
for evaluating the functional limitations resulting from immune 
system disorders: 114.02B, for systemic lupus erythematosus; 
114.03B, for systemic vasculitis; 114.04D, for systemic sclerosis 
(scleroderma); 114.05E, for polymyositis and dermatomyositis; 
114.06B, for undifferentiated and mixed connective tissue disease; 
114.07C, for immune deficiency disorders, excluding HIV infection; 
114.08L, for HIV infection; 114.09D, for inflammatory arthritis; and 
114.10B, for Sjogren's syndrome.
    2. When we use one of the listings cited in 114.00I1, we will 
consider all relevant information in your case record to determine 
the full impact of your immune system disorder on your ability to 
function. Important factors we will consider when we evaluate your 
functioning under these listings include, but are not limited to: 
Your symptoms, the frequency and duration of manifestations of your 
immune system disorder, periods of exacerbation and remission, and 
the functional impact of your treatment, including the side effects 
of your medication.
    3. To satisfy the functional criterion in a listing, your immune 
system disorder must result in an ``extreme'' limitation in one 
domain of functioning or a ``marked'' limitation in two domains of 
functioning depending on your age. (See 112.00C for additional 
discussion of these areas of functioning and Sec. Sec.  416.924a and 
416.926a for additional guidance on the evaluation of functioning in 
children.) Functional limitation may result from the impact of the 
disease process itself on your mental functioning, physical 
functioning, or both your mental and physical functioning. This 
could result from persistent or intermittent symptoms, such as 
depression, severe fatigue, or pain, resulting in a limitation of 
your ability to do a task, to concentrate, to persevere at a task, 
or to perform the task at an acceptable rate of speed. You may also 
have limitations because of your treatment and its side effects (see 
114.00G).

J. How do we evaluate your immune system disorder when it does not 
meet one of these listings?

    1. These listings are only examples of immune system disorders 
that we consider severe enough to result in marked and severe 
functional limitations. If your impairment(s) does not meet the 
criteria of any of these listings, we must also consider whether you 
have an impairment(s) that satisfies the criteria of a listing in 
another body system.
    2. Individuals with immune system disorders, including HIV 
infection, may manifest signs or symptoms of a mental impairment or 
of another physical impairment. We may evaluate these impairments 
under any affected body system. For example, we will evaluate:
    a. Growth impairment under 100.00.
    b. Musculoskeletal involvement, such as surgical reconstruction 
of a joint, under 101.00.
    c. Ocular involvement, such as dry eye, under 102.00.
    d. Respiratory impairments, such as pleuritis, under 103.00.
    e. Cardiovascular impairments, such as cardiomyopathy, under 
104.00.
    f. Digestive impairments, such as hepatitis (including hepatitis 
C) or weight loss as a result of HIV infection that affects the 
digestive system, under 105.00.
    g. Genitourinary impairments, such as nephropathy, under 106.00.
    h. Hematologic abnormalities, such as anemia, granulocytopenia, 
and thrombocytopenia, under 107.00.
    i. Skin impairments, such as persistent fungal and other 
infectious skin eruptions, and photosensitivity, under 108.00.
    j. Neurologic impairments, such as neuropathy or seizures, under 
111.00.
    k. Mental disorders, such as depression, anxiety, or cognitive 
deficits, under 112.00.
    l. Allergic disorders, such as asthma or atopic dermatitis, 
under 103.00 or 108.00 or under the criteria in another affected 
body system.
    m. Syphilis or neurosyphilis under the criteria for the affected 
body system, for example, 102.00 Special senses and speech, 104.00 
Cardiovascular system, or 111.00 Neurological.
    3. If you have a severe medically determinable impairment(s) 
that does not meet a listing, we will determine whether your 
impairment(s) medically equals a listing. (See Sec.  416.926.) If it 
does not, we will also consider whether you have an impairment(s) 
that functionally equals the listings. (See Sec.  416.926a.) We use 
the rules in Sec.  416.994a when we decide whether you continue to 
be disabled.
    114.01 Category of Impairments, Immune System Disorders.
    114.02 Systemic lupus erythematosus. As described in 114.00D1. 
With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    B. Any other manifestation(s) of SLE resulting in one of the 
following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12; or

    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.
    114.03 Systemic vasculitis. As described in 114.00D2. With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    B. Any other manifestation(s) of systemic vasculitis resulting 
in one of the following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12; or
    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.
    114.04 Systemic sclerosis (scleroderma). As described in 
114.00D3. With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    B. With one of the following:
    1. Toe contractures or fixed deformity of one or both feet, 
resulting in the inability to ambulate effectively as defined in 
114.00C6; or
    2. Finger contractures or fixed deformity in both hands, 
resulting in the inability to perform fine and gross movements 
effectively as defined in 114.00C7; or
    3. Atrophy with irreversible damage in one or both lower 
extremities, resulting in the inability to ambulate effectively as 
defined in 114.00C6; or
    4. Atrophy with irreversible damage in both upper extremities, 
resulting in the inability to perform fine and gross movements 
effectively as defined in 114.00C7.

or

    C. Raynaud's phenomenon, characterized by:
    1. Gangrene involving at least two extremities; or
    2. Ischemia with ulcerations of toes or fingers, resulting in 
the inability to ambulate effectively or to perform fine and gross 
movements effectively as defined in 114.00C6 and 114.00C7;
or

    D. Any other manifestation(s) of systemic sclerosis 
(scleroderma) resulting in one of the following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12; or
    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.
    114.05 Polymyositis and dermatomyositis. As described in 
114.00D4. With:

[[Page 14615]]

    A. Proximal limb-girdle (pelvic or shoulder) muscle weakness, 
resulting in inability to ambulate effectively or inability to 
perform fine and gross movements effectively as defined in 114.00C6 
and 114.00C7.
or

    B. Impaired swallowing (dysphagia) with aspiration due to muscle 
weakness.
or

    C. Impaired respiration due to intercostal and diaphragmatic 
muscle weakness.
or

    D. Diffuse calcinosis with limitation of joint mobility or 
intestinal motility.
or

    E. Any other manifestation(s) of polymyositis or dermatomyositis 
resulting in one of the following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12;
or

    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.
    114.06 Undifferentiated and mixed connective tissue disease. As 
described in 114.00D5. With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).
or

    B. Any other manifestation(s) of undifferentiated or mixed 
connective tissue disease resulting in one of the following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12; or

    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.
    114.07 Immune deficiency disorders, excluding HIV infection. As 
described in 114.00E. With:
    A. One or more of the following infections. The infection(s) 
must either be resistant to treatment or require hospitalization or 
intravenous treatment three or more times in a 12-month period.
    1. Sepsis; or
    2. Meningitis; or
    3. Pneumonia; or
    4. Septic arthritis; or
    5. Endocarditis; or
    6. Sinusitis documented by appropriate medically acceptable 
imaging.

or

    B. Stem cell transplantation as described under 114.00E3. 
Consider under a disability until at least 12 months from the date 
of transplantation. Thereafter, evaluate any residual impairment(s) 
under the criteria for the affected body system.
or

    C. Any other manifestation(s) of an immune deficiency disorder 
resulting in one of the following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12; or
    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.
    114.08 Human immunodeficiency virus (HIV) infection. With 
documentation as described in 114.00F and one of the following:
    A. Bacterial infections:
    1. Mycobacterial infection (for example, caused by M. avium-
intracellulare, M. kansasii, or M. tuberculosis) at a site other 
than the lungs, skin, or cervical or hilar lymph nodes, or pulmonary 
tuberculosis resistant to treatment; or
    2. Nocardiosis; or
    3. Salmonella bacteremia, recurrent non-typhoid; or
    4. In a child less than 13 years of age, multiple or recurrent 
pyogenic bacterial infections (sepsis, pneumonia, meningitis, bone 
or joint infection, or abscess of an internal organ or body cavity, 
but not otitis media or superficial skin or mucosal abscesses) 
occurring two or more times in 2 years (for children age 13 and 
older, see 114.00F4c); or
    5. Multiple or recurrent bacterial infections, including pelvic 
inflammatory disease, requiring hospitalization or intravenous 
antibiotic treatment three or more times in a 12-month period.

or

    B. Fungal infections:
    1. Aspergillosis; or
    2. Candidiasis involving the esophagus, trachea, bronchi, or 
lungs, or at a site other than the skin, urinary tract, intestinal 
tract, or oral or vulvovaginal mucous membranes; or
    3. Coccidioidomycosis, at a site other than the lungs or lymph 
nodes; or
    4. Cryptococcosis, at a site other than the lungs (for example, 
cryptococcal meningitis); or
    5. Histoplasmosis, at a site other than the lungs or lymph 
nodes; or
    6. Mucormycosis; or
    7. Pneumocystis pneumonia or extrapulmonary Pneumocystis 
infection.
or

    C. Protozoan or helminthic infections:
    1. Cryptosporidiosis, isosporiasis, or microsporidiosis, with 
diarrhea lasting for 1 month or longer; or
    2. Strongyloidiasis, extra-intestinal; or
    3. Toxoplasmosis of an organ other than the liver, spleen, or 
lymph nodes.

or

    D. Viral infections:
    1. Cytomegalovirus disease (documented as described in 
114.00F3b(ii)) at a site other than the liver, spleen, or lymph 
nodes; or
    2. Herpes simplex virus causing:
    a. Mucocutaneous infection (for example, oral, genital, 
perianal) lasting for 1 month or longer; or
    b. Infection at a site other than the skin or mucous membranes 
(for example, bronchitis, pneumonitis, esophagitis, or 
encephalitis); or
    c. Disseminated infection; or
    3. Herpes zoster:
    a. Disseminated; or
    b. With multidermatomal eruptions that are resistant to 
treatment; or
    4. Progressive multifocal leukoencephalopathy.

or

    E. Malignant neoplasms:
    1. Carcinoma of the cervix, invasive, FIGO stage II and beyond; 
or
    2. Kaposi's sarcoma with:
    a. Extensive oral lesions; or
    b. Involvement of the gastrointestinal tract, lungs, or other 
visceral organs; or
    3. Lymphoma (for example, primary lymphoma of the brain, 
Burkitt's lymphoma, immunoblastic sarcoma, other non-Hodgkin's 
lymphoma, Hodgkin's disease); or
    4. Squamous cell carcinoma of the anal canal or anal margin.

or

    F. Conditions of the skin or mucous membranes (other than 
described in B2, D2, or D3, above), with extensive fungating or 
ulcerating lesions not responding to treatment (for example, 
dermatological conditions such as eczema or psoriasis, vulvovaginal 
or other mucosal Candida, condyloma caused by human Papillomavirus, 
genital ulcerative disease).

or

    G. Neurological manifestations of HIV infection (for example, 
HIV encephalopathy, peripheral neuropathy) resulting in one of the 
following:
    1. Loss of previously acquired, or marked delay in achieving, 
developmental milestones or intellectual ability (including the 
sudden onset of a new learning disability);

or

    2. Impaired brain growth (acquired microcephaly or brain 
atrophy--see 114.00F4b); or
    3. Progressive motor dysfunction affecting gait and station or 
fine and gross motor skills.

or

    H. Growth disturbance, with:
    1. An involuntary weight loss (or failure to gain weight at an 
appropriate rate for age) resulting in a fall of 15 percentiles from 
an established growth curve (on standard growth charts) that 
persists for 2 months or longer; or
    2. An involuntary weight loss (or failure to gain weight at an 
appropriate rate for age) resulting in a fall to below the third 
percentile from an established growth curve (on standard growth 
charts) that persists for 2 months or longer; or
    3. Involuntary weight loss of 10 percent or more of baseline 
(computed based on pounds, kilograms, or body mass index (BMI)) that 
persists for 2 months or longer.

or

    I. Diarrhea, lasting for 1 month or longer, resistant to 
treatment and requiring intravenous hydration, intravenous 
alimentation, or tube feeding.

or


[[Page 14616]]


    J. Lymphoid interstitial pneumonia/pulmonary lymphoid 
hyperplasia (LIP/PLH complex), with respiratory symptoms that 
significantly interfere with age-appropriate activities, and that 
cannot be controlled by prescribed treatment.

or

    K. One or more of the following infections (other than described 
in A-J, above). The infection(s) must either be resistant to 
treatment or require hospitalization or intravenous treatment three 
or more times in a 12-month period.
    1. Sepsis; or
    2. Meningitis; or
    3. Pneumonia; or
    4. Septic arthritis; or
    5. Endocarditis; or
    6. Sinusitis documented by appropriate medically acceptable 
imaging.

or

    L. Any other manifestation(s) of HIV infection, including those 
listed in 114.08A-K, but without the requisite findings for those 
listings (for example, oral candidiasis not meeting the criteria in 
114.08F, diarrhea not meeting the criteria in 114.08I), or other 
manifestation(s) (for example, oral hairy leukoplakia, 
hepatomegaly), resulting in one of the following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12; or
    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.
    114.09 Inflammatory arthritis. As described in 114.00D6. With:
    A. Persistent inflammation or persistent deformity of:
    1. One or more major peripheral weight-bearing joints resulting 
in the inability to ambulate effectively (as defined in 114.00C6); 
or
    2. One or more major peripheral joints in each upper extremity 
resulting in the inability to perform fine and gross movements 
effectively (as defined in 114.00C7).

or

    B. Inflammation or deformity in one or more major peripheral 
joints with:
    1. Involvement of two or more organs/body systems with one of 
the organs/body systems involved to at least a moderate level of 
severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

or

    C. Ankylosing spondylitis or other spondyloarthropathies, with:
    1. Ankylosis (fixation) of the dorsolumbar or cervical spine as 
shown by appropriate medically acceptable imaging and measured on 
physical examination at 45[deg] or more of flexion from the vertical 
position (zero degrees); or
    2. Ankylosis (fixation) of the dorsolumbar or cervical spine as 
shown by appropriate medically acceptable imaging and measured on 
physical examination at 30[deg] or more of flexion (but less than 
45[deg]) measured from the vertical position (zero degrees), and 
involvement of two or more organs/body systems with one of the 
organs/body systems involved to at least a moderate level of 
severity.

or

    D. Any other manifestation(s) of inflammatory arthritis 
resulting in one of the following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12; or
    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.
    114.10 Sj[ouml]gren's syndrome. As described in 114.00D7. With:
    A. Involvement of two or more organs/body systems, with:
    1. One of the organs/body systems involved to at least a 
moderate level of severity; and
    2. At least two of the constitutional symptoms or signs (severe 
fatigue, fever, malaise, or involuntary weight loss).

OR

    B. Any other manifestation(s) of Sj[ouml]gren's syndrome 
resulting in one of the following:
    1. For children from birth to attainment of age 1, at least one 
of the criteria in paragraphs A-E of 112.12; or
    2. For children age 1 to attainment of age 3, at least one of 
the appropriate age-group criteria in paragraph B1 of 112.02; or
    3. For children age 3 to attainment of age 18, at least two of 
the appropriate age-group criteria in paragraph B2 of 112.02.

 [FR Doc. E8-5023 Filed 3-17-08; 8:45 am]
BILLING CODE 4191-02-P