[Federal Register Volume 71, Number 150 (Friday, August 4, 2006)]
[Proposed Rules]
[Pages 44432-44464]
From the Federal Register Online via the Government Publishing Office [www.gpo.gov]
[FR Doc No: 06-6655]



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Part IV





Social Security Administration





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20 CFR Part 404



Revised Medical Criteria for Evaluating Immune System Disorders; 
Proposed Rule

  Federal Register / Vol. 71, No. 150 / Friday, August 4, 2006 / 
Proposed Rules  

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SOCIAL SECURITY ADMINISTRATION

20 CFR Part 404

RIN 0960-AF33


Revised Medical Criteria for Evaluating Immune System Disorders

AGENCY: Social Security Administration.

ACTION: Proposed rule.

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SUMMARY: We propose to revise 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 proposed revisions reflect our adjudicative 
experience, as well as advances in medical knowledge, treatment, and 
methods of evaluating immune system disorders.

DATES: To be sure your comments are considered, we must receive them by 
October 3, 2006.

ADDRESSES: You may give us your comments by: using our Internet 
facility (i.e., Social Security Online) at http://policy.ssa.gov/erm/rules.nsf/Rules+Open+To+Comment or the Federal eRulemaking Portal at 
http://www.regulations.gov; e-mail to [email protected]; telefax to 
(410) 966-2830; or, letter to the Commissioner of Social Security, P.O. 
Box 17703, Baltimore, MD 21235-7703. You may also deliver them to the 
Office of Regulations, Social Security Administration, 107 Altmeyer 
Building, 6401 Security Boulevard, Baltimore, MD 21235-6401, between 8 
a.m. and 4:30 p.m. on regular business days. Comments are posted on our 
Internet site, or you may inspect them physically on regular business 
days by making arrangements with the contact person shown in this 
preamble.

FOR FURTHER INFORMATION CONTACT: Greg Zwitch, SSA Regulations Officer, 
Office of Regulations, Social Security Administration, 107 Altmeyer 
Building, 6401 Security Boulevard, Baltimore, Maryland 21235-6401, 
(410) 965-1887 or TTY (410) 966-5609. 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 Access: 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. It is also 
available on the Internet site for SSA (i.e., Social Security Online) 
at http://policy.ssa.gov/pnpublic.nsf/LawsRegs.

What programs would these proposed regulations affect?

    These proposed regulations would affect disability determinations 
and decisions that we make for you 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 proposed regulations would 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 determinable impairment(s) as described above that
   If you file a claim under * * *       And you are * * *                                          results in * * *
--------------------------------------------------------------------------------------------------------------------------------------------------------
Title II............................  an adult or a child....  the inability to do any substantial gainful activity (SGA).
Title XVI...........................  a person age 18 or       the inability to do any SGA.
                                       older.
Title XVI...........................  a person under age 18..  marked and severe functional limitations.
--------------------------------------------------------------------------------------------------------------------------------------------------------

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 impairments that we consider severe enough to result in 
``marked and severe functional limitations.'' Although we publish the 
listings only in appendix 1 to subpart P of part 404 of our rules, 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 a person 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 a person under age 18, we first use the criteria in part 
B of the listings. If the listings in part B do not apply, and if the 
specific disease process(es) has a similar effect on adults and 
children, we then use the criteria in part A. (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. (See Sec. Sec.  404.1526 and 416.926.)
    We use the listings only to decide that you are disabled or that 
you are still disabled. We will never 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'' that we use to evaluate all disability claims. (See 
Sec. Sec.  404.1520, 416.920, and 416.924.)
    Also, when we conduct reviews to determine whether your disability 
continues, we will not find that your disability has ended based only 
on any changes in the listings. 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 the listings. In these cases, we determine 
whether you have experienced medical improvement and,

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if so, whether the medical improvement is related to the ability to 
work. If your condition(s) 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 after we decide that you have experienced medical 
improvement in your condition(s). See Sec.  416.994a(b)(2).

Why are we proposing to revise the listings for immune system 
disorders?

    We are proposing these revisions to update the listings and to 
provide more information about how we evaluate immune system disorders. 
We have not updated these rules since we first published them 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 also published final rules in the 
Federal Register adding listings 14.09 and 114.09, for inflammatory 
arthritis, to these body system listings, including introductory text 
to those listings in sections 14.00B6 and 114.00E (66 FR 58009). We 
published minor technical changes to these body system listings on 
February 24, 2002 (67 FR 20018).

How did we develop these proposed rules?

    These proposed rules reflect our adjudicative experience and 
advances in medical knowledge, treatment, and methods of evaluating 
immune system disorders. They also reflect comments we asked you to 
provide to help us develop the proposals.
    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 comments in this notice, we read and considered them carefully 
and are proposing changes in our rules based on some of the suggestions 
we received.
    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 Hearings and Appeals. Several of the changes we propose in 
these rules are based on information we obtained at these conferences.

When will we start to use these rules?

    We will not use these proposed rules until we evaluate the public 
comments we receive on them, determine whether they should be issued as 
final rules, and issue final rules in the Federal Register. If we 
publish final rules, we will explain in the preamble how we will apply 
them, and we will summarize and respond to the public comments. Until 
the effective date of any final rules, we will continue to use our 
current rules.

How long would these proposed rules be effective?

    If we publish these proposed rules as final rules, they will remain 
in effect for 8 years after the date they become effective, unless we 
extend them, or revise and issue them again.

What revisions are we proposing to make?

    We are proposing to:
     Expand and reorganize the introductory text in proposed 
14.00 and 114.00 to provide more guidance for our adjudicators, to 
update it, and to reflect the revised listings.
     Add paragraph headings to the introductory text in 
proposed 14.00 and 114.00 for easier reference.
     Add proposed 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, current listing 14.08G1 for human immunodeficiency virus 
(HIV) infection with anemia is a reference listing that requires 
evaluation under current listing 7.02 for chronic anemia. Therefore, it 
is redundant. Instead of using a reference listing, we propose to 
provide general guidance in the introductory text to the immune system 
listings (proposed 14.00J2g) stating that hematologic abnormalities, 
such as anemia, may be evaluated under 7.00ff. In some cases, we are 
also replacing reference listings with new specific listing criteria 
for the impairments. For example, current listing 14.06, for 
undifferentiated connective tissue disorders, is entirely a reference 
listing. In the proposed rules, we are replacing the reference listing 
criterion with criteria that are specific to these disorders.
     Add proposed listings 14.10 and 114.10 for evaluating 
Sjo[ouml]gren's syndrome.
     Add criteria to the listings, similar to those in current 
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 proposing to change the introductory text to the adult 
immune system listings?

    We propose to expand and reorganize the introductory text to these 
listings. There are four major sections in current 14.00, and the 
longest of those sections, 14.00D, addresses only the evaluation of HIV 
infection. In these proposed rules, we add more sections and expand the 
guidance we provide about evaluating other kinds of immune system 
disorders.
    Some of the guidance in current 14.00D is useful for evaluating 
other kinds of immune system disorders in addition to HIV infection. We 
are proposing to move that guidance from current 14.00D to new sections 
that would have more general applicability to immune system disorders. 
We are not proposing to remove any substantive guidance about how we 
evaluate HIV infection, only to reorganize some of the

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information now in 14.00D of the current rules and to give it broader 
applicability where appropriate. We are also proposing to update and 
expand some of the guidance we provide for evaluating HIV infection and 
its effects, as we describe in more detail below.
    The four sections in the current rules are:
     Current 14.00A, a short paragraph that describes generally 
the kinds of disorders we include in this body system.
     Current 14.00B, a lengthy section that discusses the 
evaluation of connective tissue disorders; that is, autoimmune 
disorders. It includes six undesignated paragraphs that primarily 
explain the kinds of evidence we need to document the existence and 
severity of these disorders, including how we evaluate loss of 
function. These paragraphs are followed by six numbered sections that 
provide guidance about specific impairments in the listings.
     Current 14.00C, a single sentence that explains that we 
evaluate allergic disorders under the appropriate listing of the 
affected body system.
     Current 14.00D, a lengthy section that explains how we 
document the existence and severity of HIV infection, including how we 
evaluate loss of function under listing 14.08N. It includes eight 
numbered subsections and many paragraphs that are not designated with 
letters or numbers within those subsections.
    In the proposed rules, there are 10 sections in the introductory 
text. The first three sections (proposed 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 (proposed 
14.00D); immune deficiency disorders, excluding HIV infection (proposed 
14.00E); and HIV infection (proposed 14.00F). The next three sections 
explain how we consider the effects of your treatment (proposed 
14.00G), your symptoms (proposed 14.00H), and the functional 
limitations from your immune system disorder under these listings 
(proposed 14.00I). The last section, proposed section 14.00J, explains 
how we consider the effects of your immune system disorder when it does 
not meet the requirements of one of the proposed immune system 
listings. We are designating all paragraphs in the proposed rules with 
letters or numbers to make it easier to refer to them. 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 proposed 
14.00. We describe each section in detail later in this preamble.
     Proposed 14.00A: What disorders do we evaluate under the 
immune system listings? 
     Proposed 14.00B: What information do we need to show that 
you have an immune system disorder? 
     Proposed 14.00C: Definitions 
     Proposed 14.00D: What are the listed autoimmune disorders 
in these listings? 
     Proposed 14.00E: How do we evaluate immune deficiency 
disorders, excluding HIV infection (14.07)? 
     Proposed 14.00F: How do we evaluate human immunodeficiency 
virus (HIV) infection? 
     Proposed 14.00G: How will we consider the effect of 
treatment in evaluating your autoimmune disorder, immune deficiency 
disorder, or HIV infection? 
     Proposed 14.00H: How do we consider your symptoms, 
including your constitutional symptoms or pain? 
     Proposed 14.00I: How do we use the functional criteria in 
these listings? 
     Proposed 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 proposed changes in 
the introductory text of these proposed rules.

14.00 Immune System Disorders

    We propose to change 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.
Proposed 14.00A--What disorders do we evaluate under the immune system 
listings?
    In proposed 14.00A, we provide a brief overview of this body 
system. We explain the kinds of disorders we evaluate under the immune 
system listings and that we organize these impairments under the 
categories of ``autoimmune disorders,'' ``immune deficiency disorders, 
excluding HIV infection,'' and ``HIV infection.'' Proposed 14.00A has 
four subsections.
    We incorporate current 14.00A in the opening sentence of proposed 
14.00A1. We propose to revise the sentence, which explains the kinds of 
immune system dysfunction that immune system disorders may cause, to 
update and simplify it. In proposed 14.00A1a and 14.00A1b, we 
incorporate the first sentence in the sixth paragraph of current 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 second sentence 
of 14.001b, we propose to add ``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. Proposed 14.00A1c is a new paragraph 
that explains how we have organized immune system disorders in the 
preface (introductory text) of these listings.
    In proposed 14.00A2, Autoimmune disorders, we incorporate the first 
paragraph in current 14.00B to provide a brief description of 
autoimmune disorders. We propose to add 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 proposed 14.00D, the section of the introductory text that 
addresses autoimmune disorders in detail. We also propose to remove the 
last sentence of the first paragraph of current 14.00B, which explains 
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 does not provide useful 
adjudicative guidance. However, we do explain in proposed 14.00A1b that 
immune system disorders can cause limitation(s) that result in an 
``extreme'' loss of function.
    Proposed 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 
proposed 14.00E, the introductory section that addresses immune 
deficiency disorders in detail.
    In proposed 14.00A4, Human immunodeficiency virus (HIV) infection, 
we provide a brief description of HIV infection. We propose to move the 
first sentence in current 14.00D1 to this section. The sentence 
explains that HIV infection is caused by a specific retrovirus and may 
be characterized by increased susceptibility to opportunistic 
infections, cancers, or other conditions.

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We also provide a cross-reference to proposed 14.00F, the section of 
the introductory text that addresses HIV infection in detail.
Proposed 14.00B--What information do we need to show that you have an 
immune system disorder?
    In proposed 14.00B, we incorporate the first sentence of the second 
paragraph of current 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 current 14.00B, which define our 
term ``appropriate medically acceptable imaging,'' to proposed 14.00C, 
a new section that provides definitions of terms in these listings. We 
propose to remove the last two sentences of the current paragraph. They 
explain that we will not purchase tests that may involve significant 
risk; however, we already include this general policy in Sec. Sec.  
404.1519m and 416.919m of our regulations so it is not necessary to 
repeat them in this section.
    In the second sentence of proposed 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 include this requirement 
in current 14.00D, for HIV infection, but we do not include similar 
guidance in current 14.00B, for connective tissue disorders. We propose 
to add this guidance under proposed 14.00B because it is appropriate 
for all immune system disorders.
    We also propose to remove the third and fourth paragraphs of 
current 14.00B. The third paragraph of current 14.00B provides that we 
need a longitudinal clinical record of at least 3 months demonstrating 
active disease to assess the severity and duration of your impairment. 
However, this is not always the case, even under the current rules. For 
example, individuals with HIV infection and cryptococcal meningitis 
(current listing 14.08B4) or Kaposi's sarcoma (current listing 
14.08B8), and individuals with ankylosing spondylitis with fixation 
(ankylosis) of the dorsolumbar spine at 45[deg] (current listing 
14.09B2) are disabled based on those findings alone. In that case, 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 each case should be decided on an 
individual basis.
Proposed 14.00C--Definitions
    In proposed 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 current 14.00B. However, we propose to replace the word ``proper'' 
in the second sentence of this definition with the phrase ``generally 
accepted and consistent with the prevailing state of medical knowledge 
and clinical practice'' to more clearly explain what we mean. We also 
propose to include in this new section the definitions of the terms 
``severe'' from the sixth paragraph of current 14.00B, ``inability to 
ambulate effectively'' and ``inability to perform fine and gross 
movements effectively'' from current 14.00B6b, and ``resistant to 
treatment,'' ``recurrent,'' and ``disseminated'' from the second, 
third, and fourth paragraphs of current 14.00D2. All of these terms 
will apply to several, and sometimes all, of the proposed listings in 
this body system.
    In proposed 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'' in current 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 proposed change would also make 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 also propose to move and simplify the definitions of the terms 
``resistant to treatment,'' ``recurrent,'' and ``disseminated'' in 
current 14.00D2, primarily to remove language that we believe is 
unnecessary. For example, we removed the explanation that the terms 
``have the same general meaning as used by the medical community.'' 
These changes are only editorial. We do not intend the proposed 
definitions to be substantively different from the current rules.
    In proposed 14.00C8, we reference current 1.00F for the definition 
of ``major peripheral joints'' instead of restating the definition as 
we do in current 14.00B6a. We also propose to add the definitions of 
several other important terms in these listings, including in proposed 
14.00C2, the term ``constitutional symptoms or signs.'' In proposed 
14.00C2, we also provide brief definitions for the constitutional 
symptoms ``severe fatigue'' and ``malaise.'' We propose to add these 
definitions in response to the many comments we received that indicated 
that the fatigue and malaise that people who have immune system 
disorders experience can be very limiting.
Proposed 14.00D--What are the listed autoimmune disorders in these 
listings?
    In proposed 14.00D, we incorporate and expand upon the information 
in current 14.00B1 through 14.00B6, which describe 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. We 
also propose to add a new section 14.00D7 for Sj[ouml]gren's syndrome 
because we are proposing to add new listing 14.10 for that autoimmune 
disorder.
    In proposed 14.00D1, Systemic lupus erythematosus (14.02), we 
expand and clarify the information in current 14.00B1. In proposed 
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 that may be involved. We expand our 
explanation of how SLE is frequently characterized clinically and 
propose to change ``fatigability'' used in current 14.00B1 to 
``fatigue'' to be consistent with how we describe this symptom 
throughout the immune system listings. We also add ``involuntary'' as a 
descriptor of weight loss to clarify that we mean weight loss due to 
SLE or its treatment. In proposed 14.00D1b, Documentation of SLE, we 
propose to update 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 current rules.
    In proposed 14.00D2, Systemic vasculitis (14.03), we clarify the 
information in the current rule. Proposed 14.00D2a, General, 
corresponds to the first three sentences of current 14.00B2. In it, we 
explain that vasculitis is an inflammation of blood vessels that may 
occur acutely in association with adverse drug reactions, certain 
chronic infections, and

[[Page 44436]]

occasionally malignancies, and that it may also be associated with 
other autoimmune disorders. We also give examples of several clinical 
patterns in which it may occur. We propose to remove the fourth 
sentence of current 14.00B2, which describes 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.
    Proposed 14.00D2b, Documentation of systemic vasculitis, 
corresponds to the last two sentences of current 14.00B2. In it, we 
describe documentation that we use to confirm the diagnosis of systemic 
vasculitis.
    Proposed 14.00D3, Systemic sclerosis (scleroderma) (14.04), 
corresponds to current 14.00B3. We propose to revise the heading and to 
expand the information in the section. Proposed 14.00D3a, General, 
corresponds to the first three sentences of current 14.00B3. We propose 
to change the term ``Raynaud's phenomena,'' which we use in the second 
and third sentences of current 14.00B3, to ``Raynaud's phenomenon'' 
because the latter is the correct term. We make this same change in 
proposed listing 14.04C. In proposed 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 the current rule.
    Proposed 14.00D3c, Localized scleroderma (linear scleroderma or 
morphea), is new. We propose to add this section and appropriate 
listings in proposed 14.04 for these disorders that originate in 
childhood because their disabling effects can persist into adulthood. 
Proposed 14.00D3c is essentially the same as proposed 114.00D3c, which 
we describe in detail later in this preamble.
    Proposed 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 proposed 14.00D4, Polymyositis and dermatomyositis (14.05), we 
clarify the information in current 14.00B4. Proposed 14.00D4a, General, 
corresponds to the first three sentences of current 14.00B4. It 
describes the characteristics of polymyositis and dermatomyositis. In 
proposed 14.00D4b, Documentation of polymyositis or dermatomyositis, we 
describe the findings that are generally used to document these 
impairments. The first sentence of the proposed rule corresponds to the 
last sentence of current 14.00B4. We propose minor editorial revisions, 
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 propose to 
add a sentence to explain that people with dermatomyositis have a 
characteristic skin rash.
    In proposed 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. Proposed 14.00D4c(i) corresponds to the fourth and fifth 
sentences of current 14.00B4. We propose to delete the example of 
weakness of the anterior neck flexor muscles in the sixth sentence of 
current 14.00B4 because we are proposing to delete the reference to the 
cervical muscles from listing 14.05 for reasons we explain later in 
this preamble. We also propose to add an example of squatting. 
Squatting is a common means for evaluating weakness in the pelvic 
girdle muscles.
    In proposed 14.00D4c(ii), we explain that we will evaluate 
malignancies (which may be associated with these disorders) under the 
malignant neoplastic diseases listings (13.00ff). We do not provide 
this guidance in proposed 114.00D4c in the 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 proposed 14.00D5, Undifferentiated and mixed connective tissue 
disease (14.06), we reorganize and clarify the information in current 
14.00B5. In the proposed rules, we are adding an explicit reference to 
mixed connective tissue disease (MCTD) to clarify what we mean in the 
current rules when we refer to ``overlap'' syndromes. This is not a 
substantive change, but a clarification of our current rules to update 
medical terminology. In proposed 14.00D5a, General, we describe what we 
mean by undifferentiated and mixed connective tissue disease. In 
proposed 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 proposed 14.00D5a and 
14.00D5b are not substantively different from the provisions in the 
first three sentences of current 14.00B5.
    We propose to delete the last sentence of current 14.00B5. The 
current sentence indicates that the correct designation of an 
``overlap'' disorder is important for the assessment of prognosis. We 
believe that this sentence, while useful in treatment settings, does 
not provide useful adjudicative guidance.
    In proposed 14.00D6, Inflammatory arthritis (14.09), we expand, 
reorganize, and clarify the rules in current 14.00B6. Proposed 
14.00D6a, General, corresponds to the first and fourth sentences of 
current 14.00B6. We continue to explain that inflammatory arthritides 
include a vast array of disorders that differ in cause, course, and 
outcome and may result in difficulties of ambulation or fine and gross 
movements. We edited the fourth sentence of current 14.00B6 to break it 
up into three shorter sentences. However, we do not intend to change 
the meaning of the provision.
    Proposed 14.00D6b, Inflammatory arthritides involving the axial 
spine (spondyloarthropathies), and 14.00D6c, Inflammatory arthritides 
involving the peripheral joints, correspond to the second and third 
sentences of current 14.00B6. In these sections, we list some disorders 
that may be associated with inflammatory spondyloarthropathies 
involving the axial spine (proposed 14.00D6b) and inflammatory 
arthritides affecting the peripheral joints (proposed 14.00D6c). We 
propose to add inflammatory bowel disease (IBD) to the lists of 
examples in both sections because arthritis is the most common extra-
intestinal complication of IBD. In proposed 14.00D6b, we remove the 
examples of ``other reactive arthropathies'' and ``undifferentiated 
spondylitis'' now included in the second sentence of current 14.00D6 
because they are non-specific and the list is not intended to be 
complete, only to provide some examples. Finally, we propose to update 
some of the terminology in this section; for example, we refer to 
``psoriatic arthritis'' instead of ``psoriatic arthropathy.''
    Proposed 14.00D6d, Documentation of inflammatory arthritides, is 
new. In it, we explain that generally, but not always, the diagnosis of 
inflammatory arthritis is made by the clinical features and serologic 
findings described in the most recent edition of the Primer on the 
Rheumatic Diseases.
    Proposed 14.00D6e, How we evaluate the inflammatory arthritides 
under the

[[Page 44437]]

listings, corresponds to the information in the last two sentences of 
current 14.00B6, current 14.00B6c, and current 14.00B6d. We are 
reorganizing the text to reflect the proposed reorganization of listing 
14.09, which we explain later in this preamble, and to clarify it.
     Proposed 14.00D6e(i) explains that proposed listings 
14.09A and 14.09C1 (current 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, would only be a 
clarification of the current rule. In the proposed rule, we retain the 
provision from current 14.00B6c that the inability to ambulate 
effectively is implicit in proposed listing 14.09C1 (current 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.
     Proposed 14.00D6e(ii) explains proposed listings 14.09B 
(current listing 14.09D), 14.09C2 (current listing 14.09E), and 14.09D. 
These listings do not describe a single impairment manifestation that 
results in an ``extreme'' limitation. Rather, they describe 
combinations of impairment manifestations that should result in an 
``extreme'' limitation or in ``marked'' limitations in at least two 
areas of functioning. We also incorporate the provision in the first 
sentence of current 14.00B6d that extra-articular impairments may meet 
listings in other body systems.
     Proposed 14.00D6e(iii) corresponds to the third and fourth 
sentences of current 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 propose to reorganize and expand the list of examples 
of such impairments and to clarify the body systems to which they 
belong.
     Proposed 14.00D6e(iv) and 14.00D6e(v) correspond to the 
last sentence of current 14.00B6. In proposed 14.00D6e(iv), we replace 
``persistent'' with ``permanent'' and remove ``without ongoing 
inflammation'' to clarify that we evaluate permanent deformity of a 
major peripheral joint under listing 1.02 when it is the dominant 
feature of your impairment. Proposed 14.00D6e(v) explains that we use 
listing 1.03 to evaluate surgical reconstruction of a major weight-
bearing joint.
     Proposed 14.00D6e(vi) would clarify that we evaluate your 
impairment under any appropriate listing when you have both 
inflammation and chronic deformities.
    We are not including the provisions of current 14.00B6e in proposed 
14.00D6. Current 14.00B6e provides 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 58010, 58020 (2001)). We are proposing 
to remove that provision for reasons we explain below in our summary of 
the proposed rules in part B. Therefore, we are proposing to remove 
this provision in part A for consistency with that change. However, in 
proposed 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 remember to consider 
the side effects an individual might experience from steroids and any 
other treatment.
    Proposed 14.00D7, Sj[ouml]gren's syndrome (14.10), is new. As 
already noted, we are proposing to add a new listing for Sj[ouml]gren's 
syndrome. In connection with that proposed listing, proposed 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 proposed 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 ``Criteria 
for the Classification of Sj[ouml]gren's Syndrome'' found in the most 
recent edition of the Primer on the Rheumatic Diseases.
Proposed 14.00E--How do we evaluate immune deficiency disorders, 
excluding HIV infection (14.07)?
    In proposed 14.00E, we add a new section describing how immune 
deficiency disorders (excluding HIV infection) are classified, 
documented, and evaluated. This section has four subsections.
    In proposed 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 proposed 14.00E2, Documentation of immune deficiency disorders, 
we explain that documentation of these disorders may be made by 
laboratory evidence or by other generally acceptable methods consistent 
with the prevailing state of medical knowledge and clinical practice.
    In proposed 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 proposed 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 13.00L4 of those listings, 
we also included a special provision for autologous bone marrow 
transplants--transplants using your own stem cells (69 FR 67034). We do 
not include such an alternative provision in these proposed rules 
because people with immune deficiency disorders receive allogeneic 
transplants--that is, stem cells taken from other people. Also, we 
propose to use ``stem cell transplantation'' instead of ``bone marrow 
or stem cell transplantation'' in this proposed section and in proposed 
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 proposed 14.00E3b, Evaluation after the 12-
month period has elapsed, we explain that, after that period has 
elapsed, we consider any demonstrable residuals of your immune 
deficiency disorder including any residual impairment(s) resulting from 
your treatment. The provision also is based on 13.00L4 in our malignant 
neoplastic diseases listings.
    Proposed 14.00E4, Medication-induced immune suppression, is new. We 
explain that medication effects 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.

[[Page 44438]]

Proposed 14.00F--How do we evaluate human immunodeficiency virus (HIV) 
infection?
    In proposed 14.00F, we incorporate, update, and expand information 
on HIV infection contained in current 14.00D3 through 14.00D7. We also 
make nonsubstantive editorial changes.
    As already noted, we propose to move the first sentence of current 
14.00D1 to proposed 14.00A4. Therefore, we begin proposed 14.00F with 
what is now the second sentence of current 14.00D1. It is a reminder 
that an individual with HIV infection need not meet the Centers for 
Disease Control definition of acquired immune deficiency syndrome 
(AIDS) to meet or medically equal the criteria of listing 14.08. We 
have made minor editorial changes to the sentence, but we do not intend 
to change its meaning.
    We propose to move the provisions of current 14.00D2 to other 
sections in the proposed rules. In the first four paragraphs of current 
14.00D2, we define the terms ``resistant to treatment,'' ``recurrent,'' 
and ``disseminated,'' and we would now define those terms in proposed 
14.00C. In the fifth paragraph of current 14.00D2, we define 
``significant involuntary weight loss'' for purposes of current listing 
14.08I (which has become listing 14.08H in these proposed rules). In 
the proposed rules, we include this definition in 14.00F5.
    Like current 14.00D3, proposed 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 proposed 14.00F1, Documentation of HIV infection, we 
incorporate and update the information in current 14.00D3 to explain 
the laboratory tests or other evidence we accept as documentation of 
HIV infection. Proposed 14.00F1a, Documentation of HIV infection by 
definitive diagnosis, corresponds to current 14.00D3a. We propose to 
update and expand this section to include newer laboratory diagnostic 
techniques that did not exist or were not widely used when we published 
the current rules in 1993.
     Proposed 14.00F1a(i), for HIV antibody tests, corresponds 
to current 14.00D3a(i). We propose only nonsubstantive editorial 
changes.
     Proposed 14.00F1a(ii) is new. It would add 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 current rules.
     Proposed 14.00F1a(iii) is for HIV DNA detection by 
polymerase chain reaction (PCR). We include it as an example of an 
``other test'' in current 14.00D3a(iii) because it was not widely 
available when we published the current rules.
     Proposed 14.00F1a(iv), for HIV antigen, corresponds to 
current 14.00D3a(ii).
     Proposed 14.00F1a(v) is new. It would add 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.
     Proposed 14.00F1a(vi), for other tests that are highly 
specific for detection of HIV, corresponds to the first paragraph in 
current 14.00D3a(iii).
    Proposed 14.00F1b, Other acceptable documentation of HIV infection, 
corresponds to current 14.00D3b. It explains what documentation of HIV 
infection we will accept instead of definitive laboratory testing. The 
proposed rule is essentially the same as the current rule except for 
nonsubstantive editorial changes.
    In proposed 14.00F2, CD4 tests, we combine the provisions in the 
second undesignated paragraph after current 14.00D3a(iii) and the 
second paragraph in current 14.00D4a. We specify that, even though a 
reduced CD4 count or percent alone does not establish a definitive 
diagnosis of HIV infection, a CD4 count below 200/mm\3\ or 14 percent 
along with clinical findings does offer supportive evidence of 
opportunistic infections without a definitive diagnosis. This is 
because a CD4 count below 200 or 14 percent is an indicator of an 
increased susceptibility to developing opportunistic infections. We 
also make nonsubstantive editorial changes.
    In proposed 14.00F3, Documentation of the manifestations of HIV 
infection, we incorporate the information in current 14.00D4 with 
nonsubstantive editorial changes. Like proposed 14.00F1 and current 
14.00D4, proposed 14.00F3 is divided into two main parts. The first 
section explains how we document manifestation of HIV infection 
definitively (14.00F3a), and the second section explains how we 
document manifestations of HIV infection when we do not have definitive 
evidence (14.00F3b).
    Proposed 14.00F3a, Documentation of the manifestations of HIV by 
definitive diagnosis, incorporates the first paragraph in current 
14.00D4a.
    In proposed 14.00F3b, Other acceptable documentation of the 
manifestations of HIV infection, we incorporate information that is in 
the first paragraph of current 14.00D4b. We propose to revise the 
language of this paragraph both editorially and to clarify our original 
intent. In the current rule, we indicate 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 
imply that we need 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 is not our intent, so we are 
clarifying in the proposed 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 also propose to clarify what we mean by 
``laboratory findings'' in this context; that is, laboratory findings 
that do not in themselves definitively establish the existence of a 
diagnosis of an HIV-related manifestation.
    In 14.00D4 of the current rules we provide specific guidance for 
documenting one particular manifestation of HIV infection without 
definitive evidence: cytomegalovirus (CMV) disease. In proposed 
14.00F3b, we expand the section to include two additional 
manifestations. In proposed 14.00F3b(i), we add guidance to explain 
that Pneumocystis carinii pneumonia (PCP) is frequently diagnosed 
presumptively without definitive evidence and to provide examples of 
evidence that is supportive of a presumptive diagnosis of PCP. We also 
note that Pneumocystis carinii is now known as Pneumocystis jiroveci; 
however, ``PCP'' remains in common usage for the pneumonia caused by 
this organism.
    In proposed 14.00F3b(ii), we incorporate and expand the information 
now in the second paragraph of current 14.00D4b, regarding the 
documentation of CMV disease. We propose to clarify that a positive 
serology test for CMV identifies a ``history'' of infection but does 
not confirm an ``active'' disease process. We do not include 
``documentation of CMV disease requires confirmation by biopsy'' as in 
the last sentence of the second

[[Page 44439]]

paragraph of current 14.00D4 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 
positive CMV serology test, that are supportive evidence of a 
presumptive diagnosis of CMV disease.
    In proposed 14.00F3b(iii), we add guidance on 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 proposed 14.00F4, Manifestations specific to women, we 
incorporate the information in current 14.00D5. In proposed 14.00F4a, 
General, we incorporate the first paragraph of current 14.00D5 and in 
proposed 14.00F4b, Additional considerations for evaluating HIV 
infection in women, we incorporate the second paragraph of current 
14.00D5. Except for adding paragraph designations and headings and 
minor editorial changes (including changes to reflect proposed changes 
in the paragraph designations of the listings explained below), the 
proposed provisions are the same as in the current rules.
    In proposed 14.00F5, involuntary weight loss, we incorporate the 
last paragraph of current 14.00D2 with nonsubstantive editorial 
changes, including a change that reflects our proposal to redesignate 
listing 14.08I to listing 14.08H.
Proposed 14.00G--How will we consider the effect of treatment in 
evaluating your autoimmune disorder, immune deficiency disorder, or HIV 
infection?
    In the current rules, we refer to treatment and its effects in four 
places.
     In the third paragraph of 14.00B, we provide that, for 
connective tissue diseases, we need a longitudinal clinical record of 
at least 3 months demonstrating active disease despite prescribed 
treatment, with the expectation that the disease will remain active for 
12 months.
     In the fifth paragraph of 14.00B, we explain that ``the 
chronic adverse effects of treatment (e.g., corticosteroid-related 
ischemic necrosis of bone) may result in functional loss'' in 
individuals with connective tissue disease.
     In 14.00B6e, we explain that the fact that an individual 
with inflammatory arthritis is dependent on steroids or any other drug 
for the control of the arthritis is not in itself sufficient to 
establish that the individual is disabled. We also explain that we must 
evaluate each case on its own merits, taking into consideration any 
adverse effects of treatment.
     In 14.00D7, Effect of treatment, we provide three 
paragraphs discussing how we consider treatment in people with HIV 
infection. This section explains that we must consider both the 
positive effects and negative side effects of treatment for HIV 
infection and its manifestations, special considerations in evaluating 
treatment in individuals with HIV infection and, briefly, the kinds of 
evidence we need.
    We are proposing to remove the provisions in the third paragraph of 
14.00B and paragraph 14.00B6e. Neither of those sections nor the other 
current rules we will continue to use contain provisions that explain 
in detail how we evaluate the positive effects and negative side 
effects of treatment in individuals who have autoimmune disorders and 
immune deficiency disorders apart from HIV infection. Also, most 
current treatments for HIV infection came into use, or came into wide 
use, after we first published listing 14.08 in 1993. As a consequence, 
we believe that current 14.00D7 needs to be updated to reflect the 
newer and more widely used treatments and treatment protocols for HIV 
infection and to reflect the considerable medical experience that has 
been gained since 1993 about the long-term effects, usefulness, and 
limitations of such treatments.
    Therefore, we propose to add a new separate section 14.00G--How 
will we consider the effect of treatment in evaluating your autoimmune 
disorder, immune deficiency disorder, or HIV infection? The new section 
would address 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 do not propose to remove any 
guidance about treatment for HIV infection that is still relevant, only 
to move it to this new section. In fact, we propose to expand and 
update our rules to reflect what has been learned in applying different 
treatments for HIV infection since we published the current rules more 
than a decade ago. 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 would be new in these listings and, we believe, would provide 
useful adjudicative guidance that is lacking in our current rules.
    Section 14.00G has six subsections. The first two (proposed 14.00G1 
and 14.00G2) and the last one (proposed 14.00G6) are applicable to all 
immune system disorders. Proposed 14.00G3-14.00G5 provide guidance 
specific to each of the three main types of immune system disorders: 
Autoimmune disorders (proposed 14.00G3), immune deficiency disorders, 
excluding HIV infection (proposed 14.00G4), and HIV infection (proposed 
14.00G5).
    In proposed 14.00G1, General, we incorporate the first and fifth 
sentences of current 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 both 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 mention in the current rule, but we propose to expand the 
list and in some cases to clarify the existing factors in our current 
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 (the dosing schedule, 
need for injections, etc).'' In proposed 14.00G1e, we also introduce 
the term ``variability of your response to treatment,'' a concept we 
address for HIV infection in current 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 proposed 14.00G2.
    Proposed 14.00G1f is new. It describes the interactive and 
cumulative effects of treatments for immune system disorders and other 
disorders that people 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. Proposed 14.00G1g is also new. It explains 
that we will also consider the duration of your treatment. Proposed 
14.00G1h is a catchall for other relevant factors we have not listed in 
14.00G1a-14.00G1g.
    In proposed 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

[[Page 44440]]

system disorders. This proposed rule is based on the language of the 
second paragraph in current 14.00D7 and the second sentence of the 
third paragraph of that section. However, we propose to expand that 
guidance and to apply 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 proposed 14.00G3, How we evaluate the effects of treatment 
for autoimmune disorders on your ability to function. This proposed 
rule repeats the rule in the fifth paragraph of current 14.00B that, 
when we evaluate the effects of your treatment for your autoimmune 
disorder(s), we will consider the adverse effects that may result in 
loss of function. We propose to expand 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 also propose to 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.
    Proposed 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 proposed 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 
fatigue and headaches), clinical signs (such as high blood pressure and 
joint swelling), and limitations in mental function, including 
cognition, concentration, and mood.
    Proposed 14.00G5, How we evaluate the effects of treatment for HIV 
infection on your ability to function, is in two parts. In proposed 
14.00G5a, General, as in proposed 14.00G3 and 14.00G4, we repeat the 
principle from 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 propose to expand our 
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, but that 
we will consider your functional limitations whether they are a result 
of your symptoms from HIV infection or the side effects of your 
treatment.
    In proposed 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 proposed guidance clarifies that STIs are part of a 
prescribed treatment plan and 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 proposed 14.00G6, When there is no record of ongoing treatment, 
we explain how we will 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 systems 
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 provide in the 
last sentence of the third paragraph in current 14.00D7, which explains 
we should decide the impact of treatment based on a sufficient period 
of treatment.
Proposed 14.00H--How do we consider your symptoms, including your 
constitutional symptoms or pain?
    Proposed 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.
Proposed 14.00I--How do we use the functional criteria in these 
listings?
    Although we indicated in the ANPRM that we would not summarize or 
respond to the public comments (68 FR 24897), there was one theme that 
was common to many of the letters and e-mails and that was raised 
repeatedly by the medical specialists, advocates for people who have 
immune system disorders, and individuals with immune system disorders 
in the presentations at the two outreach meetings we held: 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 people said to help 
explain why we are proposing to add new rules for evaluating 
functioning in these listings.
    Many people said that we should recognize how immune system 
disorders can affect an individual's functioning. Many people 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 people indicated that the fatigue associated with 
these disorders was not merely a feeling of tiredness but a more 
profound and debilitating experience. Many people also noted that the 
impairments could be both episodic and variable in intensity, with some 
people 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 people pointed out 
that there is often comorbidity of immune system disorders; that is, 
many people have features of more than one immune system disorder. In 
those cases, the symptoms and limitations are multiplied to an effect 
that is worse than simply adding them up. These commenters said that 
under the current listings there is no adequate way to assess these 
multiplied effects. Many people also pointed out the effect that stress 
can have on the medical condition and symptomatology of individuals who 
have immune system disorders. Other people described the debilitating 
effects of treatment, not only the side effects, but sometimes the need 
to follow a very rigorous and time-

[[Page 44441]]

consuming schedule of treatment that in itself can be limiting.
    A number of the commenters pointed with approval to the provisions 
of current listing 14.08N and the text in current 14.00D8 that explains 
that listing. These individuals thought that the provisions should not 
be confined to people who have HIV infection but should be extended to 
people 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.
    We carefully considered these comments and are proposing a number 
of changes throughout the introductory text to the immune system 
listings to address them. We are proposing to significantly expand our 
guidance about specific immune system disorders and the effects of 
treatment. We agree with those commenters who suggested that we include 
the same kind of criteria for evaluating the overall functional impact 
of other immune system disorders as we provide in current listing 
14.08N for people who have HIV infection. Therefore, we are proposing 
to add criteria similar to those in current listing 14.08N for each of 
the listed impairments in this body system. The proposed listings for 
evaluating functioning for other immune system disorders would be 
14.02B, 14.03B, 14.04D, 14.05E, 14.06B, 14.07C, 14.09D, and 14.10B. We 
are also proposing to redesignate current listing 14.08N as 14.08K for 
reasons we explain below.
    Proposed 14.00I is the section of the introductory text that would 
explain the proposed listings that include functional criteria. It 
corresponds to current 14.00D8, but we revised it so that it applies to 
all of the new proposed listings that include functional criteria, not 
just the listing for HIV infection (current listing 14.08N).
    Like current 14.00D8, proposed 14.00I includes eight paragraphs. 
Except as described below, we propose to revise 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 current 
14.00D8 we explain that current listing 14.08N (proposed listing 
14.08K) establishes standards for evaluating manifestations of HIV 
infection that do not meet the criteria of any of the preceding 
listings within 14.08; that is, current listings 14.08A-14.08M. We also 
explain that we use listing 14.08N both for manifestations that are 
listed in the preceding listings within 14.08 and for manifestations 
that are not listed at all. We propose to modify this language so that 
it applies to all of the immune system disorders within this body 
system. We also propose minor editorial changes throughout the 
paragraphs.
    The following are other changes we propose to make in this section.
    In proposed 14.00I2, we propose to remove the first sentence in the 
second paragraph of current 14.00D8, which explains that for 
individuals with HIV infection, we assess listing-level severity under 
current listing 14.08N based on the functional limitations imposed by 
the impairment. We believe that this point is already made in proposed 
14.00I1 and that it is unnecessary to repeat it in proposed 14.00I2. We 
propose to revise the second sentence, which says 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 
clearly explain what we intend. Therefore, we propose to revise it to 
explain that 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(s) on your ability to 
function on a sustained basis.
    In proposed 14.00I3-14.00I8, which correspond to the last six 
paragraphs in current 14.00D, we propose to update our rules to make 
their language more consistent with our other rules that define the 
term ``marked'' and the domains of functioning. We do not intend these 
changes to be substantively different from the current rules. We also 
propose to include references to both pain and fatigue throughout 
proposed 14.00I6-14.00I8 as symptoms that may cause limitations. The 
current rules are not consistent in this regard.
Proposed 14.00J-- How do we evaluate your immune system disorder when 
it does not meet one of these listings?
    Proposed 14.00J1 and 14.00J3 would replace the guidance we now 
provide in the first and third paragraphs of current 14.00D6. As in 
other provisions throughout the introductory text, we propose to revise 
the language to make it apply generally to all immune system disorders, 
not just HIV infection. Also, we propose to remove guidance that is 
already covered in other sections in the introductory text of these 
proposed rules, such as the guidance that individuals may have signs or 
symptoms of a mental impairment or of another physical impairment.
    Proposed 14.00J2 would be a new section in this body system. For 
reasons we explain below, we are proposing to remove 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 propose to add new 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 systems listings. The 
proposed provisions are based on language in the second paragraph of 
current 14.00D6, which is currently relevant only to the evaluation of 
HIV infection, and on the reference listings we are proposing to 
remove. In the latter case, we are also expanding the information to 
provide specific examples of impairments that may be caused by 
autoimmune disorders.
    For example, current listings 14.02A6 and 14.04A4 are 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 are unnecessary because any individual who 
meets the criteria of a listing in the digestive body system (5.00ff) 
would be disabled under that listing, the guidance is not very 
specific. Also, in the current rules, we include these criteria only 
under listing 14.02 and 14.04; however, other immune system disorders 
can have effects in the digestive system. Therefore, we provide in 
proposed 14.00J2e 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 5.00ff.
    Proposed 14.00J2k provides examples of allergic disorders 
(including skin disorders) that individuals with immune system 
disorders may have. It would replace current 14.00C.

How are we proposing to change the criteria in the listings for 
evaluating immune system impairments in adults?

14.01 Category of Impairments, Immune System Disorders

    The following is a detailed explanation of the significant changes 
in the proposed listings. Some changes are

[[Page 44442]]

common to several listings so we describe them first.
    1. We propose to remove all of the reference listings from this 
body system. Every current listing section in this body system, except 
listing 14.07, includes reference listings. Reference listings are 
listings that are met by satisfying the criteria of another listing. 
For example, current listing 14.02A1, Joint involvement, is met when 
the resulting impairment meets the criteria of any appropriate listing 
in the musculoskeletal body system, 1.00ff. Current listing 14.08G1, 
for HIV infection with anemia, requires evaluation under current 
listing 7.02. Therefore, these listings are redundant because 
impairments that meet these listings must meet the requirements of 
other listings. We are removing reference listings from all of the body 
systems as we revise them. As already noted, instead of using reference 
listings, we propose to provide guidance in 14.00J of the introductory 
text stating that we may evaluate the resulting impairment of an immune 
system disorder under any affected body system.
    2. We propose to revise current listings 14.02B, 14.03B, 14.04B, 
and 14.09D (proposed listings 14.02A, 14.03A, 14.04A, and 14.09B) as 
follows:
     We propose to remove the criterion for ``significant, 
documented'' constitutional symptoms or signs in each of these listings 
because we define the constitutional symptoms and signs in proposed 
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 current listings, except current listing 
14.09D, also requires you to have all four of the constitutional 
symptoms or signs: Severe fatigue, fever, malaise, and involuntary 
weight loss. We propose to revise this requirement to ``at least two'' 
of the constitutional symptoms or signs instead of all four, 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. We also have added 
``involuntary'' as a descriptor of weight loss in proposed listings 
14.02A, 14.03A, 14.04A, 14.05E, 14.06A, 14.07C, 14.08K, 14.09B, and 
14.10A for the same reason we explained earlier in the preamble.
     In proposed listings 14.02A, 14.03A, and 14.04A, which 
correspond to current listings 14.02B, 14.03B, and 14.04B, we propose 
to remove the reference to ``lesser involvement'' because we propose to 
remove the current 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 proposed 
14.00I, we propose to add listings based on repeated manifestations 
accompanied by functional limitations and modeled after current listing 
14.08N for each of the other immune system disorders. The proposed new 
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 (other than HIV 
infection),
     14.09D for inflammatory arthritides, 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 that do not satisfy the requisite 
findings of another listing for the specified immune system disorder,
     At least two of the constitutional symptoms or signs, and
     ``Marked'' limitation in one of three domains of 
functioning: Activities of daily living, 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 proposed 14.00I3 and by 
``marked'' in proposed 14.00I4-5.
    The following is an explanation of the other significant changes we 
propose to make. We are also proposing 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 
in the proposed rules. We do not describe all of those changes below.
Proposed Listing 14.04--Systemic sclerosis (scleroderma)
    Proposed listing 14.04B corresponds to current listing 14.04C. As 
we have already noted, we propose to expand this listing to include 
provisions for individuals who had a childhood form of the disorder as 
children and who still have listing-level functional limitations as 
adults. The proposed listing is essentially identical to proposed 
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 also propose minor clarifications in the language of the current 
listing. Current listing 14.04C describes ``[g]eneralized scleroderma 
with digital contractures.'' We propose to clarify that ``digital'' 
refers to either the toes or the fingers, and to list the effects in 
the toes separately from the effects in the fingers, in proposed 
listings 14.04B1 and 14.04B2, respectively. We also propose to remove 
the requirement for ``generalized'' scleroderma (that is, systemic 
sclerosis) because the very serious digital contractures described in 
the proposed listings would in themselves be disabling regardless of 
whether the scleroderma is generalized.
    Proposed listing 14.04C corresponds to current listing 14.04D. We 
propose to change ``Raynaud's phenomena'' in current listing 14.04D to 
``Raynaud's phenomenon'' for the same reason already described in the 
explanation of proposed 14.00D3. We propose to remove 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 fingers or toes, resulting in the inability to ambulate 
effectively or to perform fine and gross movements effectively. As in 
proposed listing 14.04B, we also propose to clarify that ``digital'' 
refers to fingers or toes.
    In proposed listing 14.04C, we also propose to revise the criteria 
in current listing 14.04D to provide a better description of listing-
level Raynaud's phenomenon. The criteria in current listing 14.04D 
require severe Raynaud's phenomenon that is characterized by digital 
ulcerations, ischemia, or gangrene. We believe that this does not 
describe an impairment that precludes any gainful activity in every 
case. Therefore, in proposed listing 14.04C1 we would provide criteria 
for Raynaud's phenomenon characterized by gangrene of a toe or finger 
in at least two extremities, or a toe and finger to indicate an 
impairment that would preclude any gainful activity. We do not propose 
to require that the gangrene result in the inability to ambulate 
effectively or to perform fine and gross movements effectively because 
the presence of gangrene of a toe or finger

[[Page 44443]]

in at least two extremities or in a toe and finger by itself is an 
indication of a very serious impairment. In proposed 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 
propose to revise the language so that ischemia and ulcerations are not 
listed as though they are separate entities, as in the current rule.
Proposed Listing 14.05--Polymyositis and Dermatomyositis
    Proposed listing 14.05A corresponds to current listing 14.05A. We 
propose to replace 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 also propose to 
change ``shoulder and/or pelvic'' muscle weakness to ``pelvic or 
shoulder'' muscle weakness because pelvic muscle weakness can result in 
the inability to ambulate effectively and shoulder muscle weakness can 
result in the inability to perform fine and gross movements 
effectively. Therefore, either one of these findings could be 
sufficient in itself to show disability and the ``and'' is unnecessary.
    Proposed listing 14.05B corresponds to current listing 14.05B1. We 
propose to remove the requirements in the opening paragraph for less 
severe limb-girdle muscle weakness than in 14.05A, associated with 
cervical muscle weakness, because impaired swallowing or impaired 
respiration may result in listing-level limitations without the 
presence of either of those findings. We also propose to remove the 
phrase ``to at least a moderate level of severity'' because the 
criterion in proposed 14.05B is of at least a moderate level of 
severity, making this language unnecessary. We propose to revise 
``impaired swallowing with dysphagia'' to ``impaired swallowing 
(dysphagia)'' because dysphagia means impaired swallowing. We propose 
to revise ``episodes of aspiration'' to ``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 
propose to replace ``cricopharyngeal weakness'' with ``muscle 
weakness'' in proposed 14.05B because impaired swallowing with 
dysphagia and aspiration may result from muscles other than the 
cricopharyngeal muscles.
    Proposed listing 14.05C corresponds to current listing 14.05B2. We 
propose to remove the requirements in the opening paragraph of current 
14.05B2 for the same reasons as in the above paragraph for proposed 
listing 14.05B.
    Proposed listing 14.05D, Diffuse calcinosis, is a new adult listing 
and has the same criteria as in proposed listing 114.05D for children, 
which we describe in detail later in this preamble. We propose to add 
this listing for individuals who had a form of the disorder as children 
and who still have listing-level functional limitations as adults.
Proposed Listing 14.06--Undifferentiated and Mixed Connective Tissue 
Disease
    We propose to change the heading of current 14.06 to update it and 
to more accurately describe the disorders we evaluate under this 
listing.
    Current listing 14.06 is entirely a reference listing, requiring 
evaluation under current listings 14.02A, 14.02B, or 14.04. We propose 
to change it to a stand-alone listing containing its own criteria. 
Proposed listing 14.06A uses the same criteria as in proposed listings 
14.02A, 14.03A, and 14.04A for involvement of two or more body systems 
to at least a moderate level of severity and at least two 
constitutional symptoms or signs. Proposed listing 14.06B incorporates 
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.
Proposed Listing 14.07--Immune Deficiency Disorders, Excluding HIV 
Infection
    We propose to change the heading of listing 14.07 to update its 
terminology and to more accurately describe the disorders we evaluate 
under this listing.
    The current listing is met with documented, recurrent severe 
infections occurring three or more times within a 5-month period. We 
propose to replace this criterion with a new, more accurate, and up-to-
date listing. The listing is in three parts.
    Proposed listing 14.07A is essentially the same as current listing 
14.08M (proposed listing 14.08J) which describes individuals with HIV 
infection whose immune systems are so compromised that they frequently 
become ill. However, unlike current listing 14.07, current listing 
14.08M provides that the infections must occur three times in a 12-
month period, not three times in only a 5-month period. Current listing 
14.08M is also more precise. It explains how severe the infections need 
to be by reference to resistance to treatment or a requirement for 
hospitalization or intravenous treatment. It also specifies six types 
of infections. We believe that the criteria in current listing 14.08M 
for people with HIV infection are equally as applicable to individuals 
with other kinds of immune deficiency disorders, and that they would be 
more inclusive than the criteria in current listing 14.07.
    Proposed listing 14.07B is new. We propose to add this listing to 
recognize that some immune system disorders are treated by stem cell 
transplantation. In proposed listing 14.07B, we state that we will 
consider you 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.
    Proposed listing 14.07C would incorporate the same functional 
criteria for the evaluation of repeated manifestations of immune 
deficiency disorders (excluding HIV infection) as in the other proposed 
listings in this body system and for the same reasons as described 
above.
Proposed Listing 14.08--Human Immunodeficiency Virus (HIV) Infection
    We do not propose any substantive changes to the criteria in 
listing 14.08. We have carefully considered the advances in treatment 
and consequent longevity that have occurred since we published the 
current rules in 1993. However, we do not believe that there has been 
sufficient progress in the treatment and control of HIV infection to 
warrant any change in these rules. Moreover, even as some problems of 
people who have HIV infection appear to be improved, new problems have 
arisen to take their place. Advances in treatment are a case in point. 
While there have been significant strides in the treatment of HIV 
infection that have improved mortality, the treatment itself is often 
disabling both in terms of its side effects and its administration. 
Many people must structure their days and nights around their 
treatment, and any lapse can have dire consequences. Some people 
respond to treatment initially but become unresponsive without warning. 
Others have only limited success with their treatments. Relatively few 
people with HIV infection are considered ``well.'' Therefore, from the 
standpoint of Social Security disability policy and efficient

[[Page 44444]]

administration of the disability programs, we have not seen sufficient 
evidence to persuade us to propose any significant changes in this 
listing.
    As already noted, we propose to remove current reference listings 
throughout this body system, including the reference listings in 
listing 14.08. This would result in the removal of several specific 
listings within 14.08 and the redesignation of some of the current 
listings; for example, current listing 14.08N would become listing 
14.08K. Where we propose to remove a reference listing, however, we 
have ensured that we provide guidance in the introductory text about 
where to evaluate the impairment. For example, current listing 14.08A4, 
for HIV infection with syphilis or neurosyphilis, is a reference 
listing that says 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 
propose to remove this reference listing, we include this same guidance 
in proposed 14.00J2l.
    We also propose to clarify some of the rules. We propose to 
reorganize the language in listing 14.08B2 to make it clearer that we 
evaluate under this listing candidiasis involving the esophagus, 
trachea, bronchi, or lungs, or at another site other than the skin, 
urinary tract, intestinal tract, or oral or vulvovaginal mucous 
membranes. We propose to move current 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 proposed listing 14.08B7.
    We propose to redesignate current listing 14.08N as proposed 
listing 14.08K. We propose to expand our guidance on manifestations we 
evaluate under proposed listing 14.08K by adding ``pancreatitis, 
hepatitis, peripheral neuropathy, glucose intolerance, muscle weakness, 
and cognitive or other mental impairments'' as new examples. We also 
expand our list of signs or symptoms by adding ``nausea, vomiting, 
headaches, or insomnia.''
    We propose minor changes to the language of the functional criteria 
in proposed listing 14.08K from the current language in listing 14.08N. 
For example, we would replace the words ``restriction'' in current 
listing 14.08N1 and ``difficulties'' in current listings 14.08N2 and 
14.08N3 with the word ``limitation'' in proposed listings 14.08K1, 
14.08K2, and 14.08K3. We propose to make this change because 
``limitation'' is a clearer term that we use throughout our rules.
Proposed Listing 14.09--Inflammatory Arthritis
    We are redesignating current listing 14.09D as proposed listing 
14.09B, current listing 14.09B as proposed listing 14.09C1, and current 
listing 14.09E as proposed listings 14.09C2 to put them in a more 
logical order. In the proposed rules, listing 14.09A would describe 
persistent inflammation or deformity of major peripheral joints that 
alone is disabling, while listing 14.09B would describe disability with 
lesser inflammation or deformity of major peripheral joints, organ 
involvement, and constitutional symptoms. Listing 14.09C would describe 
listing-level inflammatory arthritis of the spine. Proposed listing 
14.09C1 would describe disability based only on fixation (ankylosis) of 
the spine, while listing 14.09C2 would describe disability based on a 
lesser degree of ankylosis of the spine with organ involvement. 
Proposed listing 14.09D would be the same functional listing we include 
in all of the proposed immune system listings and would apply to 
inflammatory arthritis affecting any joints.
    Proposed listing 14.09A corresponds to current listing 14.09A. We 
propose to remove the requirement for a history of joint pain, 
swelling, and tenderness from this listing because it is unnecessary 
and to provide only that joint inflammation must be ``persistent.'' (We 
do refer to joint pain, swelling, and tenderness in proposed 14.00D6a.) 
Persistent joint inflammation or deformity in two or more major 
peripheral joints resulting in the inability to ambulate effectively or 
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 also propose to remove the requirement for 
``signs on current physical examination.'' We would 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. We propose to change 
``two or more major joints'' to ``two or more major peripheral joints'' 
to distinguish these joints from the joints of the spine. We define 
``major peripheral joints'' in proposed 14.00C8.
    Proposed listing 14.09B corresponds to current listing 14.09D. The 
revisions in proposed 14.09B are similar to those in proposed 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. 
Proposed 14.09B continues to require less joint involvement than in A, 
but we would no longer require ``lesser extra-articular features than 
in C'' because ``C'' refers to current reference listing 14.09C which 
we are proposing to remove. Instead, we require ``extra-articular 
features that do not satisfy the criteria of a listing.'' Proposed 
listing 14.09B1 corresponds to current listing 14.09D2 with 
nonsubstantive editorial changes to make it consistent with how we 
present this criterion throughout these listings. Proposed listing 
14.09B2 corresponds to current listing 14.09D1 except that we have 
removed the phrase ``significant, documented'' for reasons we have 
already explained. We also propose to correct an error in current 
listing 14.09D1. The explanatory abbreviation, ``e.g.'' (for example) 
in current listing 14.09D1 inaccurately indicates that the four 
constitutional symptoms or signs, that is, fatigue, fever, malaise, and 
involuntary weight loss, are only examples when they are in fact a 
complete list. Consistent with changes in other proposed listings, we 
propose to require at least two of the constitutional symptoms or signs 
because we believe that the criteria in proposed listing 14.09B are 
indicative of an impairment that precludes any gainful activity.
    Proposed listing 14.09C1 corresponds to current listing 14.09B. We 
propose to reorganize the criteria and to remove 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 proposed 
listing 14.09C1 are in themselves indicative of an impairment that 
precludes any gainful activity.
    Proposed listing 14.09C2 corresponds to current listing 14.09E. We 
propose to reorganize this listing to make it more consistent with the 
structure and

[[Page 44445]]

criteria that we use in the proposed listings for other autoimmune 
disorders. We propose to remove 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 
current listing 14.09B, and to replace it with fixation ``at 30[deg] or 
more of flexion (but less than 45[deg]).'' We believe that this would 
be a clearer and more specific criterion that would help to provide 
greater uniformity in adjudications under this listing. We propose to 
remove the phrase ``lesser extra-articular features than in C'' because 
it refers to current reference listing 14.09C, which we are proposing 
to remove. We also propose to remove the phrase ``with signs of 
unilateral or bilateral sacroiliitis'' because the criteria in the 
proposed 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 language.
Proposed Listing 14.10--Sj[ouml]gren's Syndrome
    Proposed listing 14.10 is new. We are proposing to add it in 
response to comments we received that Sjogren's syndrome is distinct 
from other immune system disorders with unique aspects that the current 
immune system listings do not address.
    Although individuals with Sjogren's syndrome can qualify under 
current listings 14.03 and 14.09, and other listings, we believe that 
it is now appropriate to list Sjogren's syndrome separately in these 
listings. We propose to use the same two listing criteria for 
establishing listing-level severity as in the other proposed listings 
for autoimmune disorders because Sjogren'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. Proposed listing 
14.10A is the same as proposed listings 14.02A, 14.03A, 14.04A, and 
14.06A, and proposed listing 14.10B is the same as proposed listings 
14.02B, 14.03B, 14.04D, 14.05E, 14.06B, and 14.09D. We also provide a 
new separate section in the introductory text that describes the unique 
features of Sjogren's syndrome, proposed 14.00D7.

What revisions are we proposing to make in the immune system disorder 
listings for children--114.00?

    As in proposed 14.00 in the adult rules, we propose to change the 
name of this body system to ``Immune System Disorders.''
    Except for minor editorial changes, we have repeated much of the 
introductory text of proposed 14.00 in the introductory text to 
proposed 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 proposed 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 proposed 
114.00, we have made an editorial change in the terms we use to 
identify the age categories of children in the introductory text of 
current 114.00 to be consistent with the terms we use in the 
introductory text of current 112.00, Mental Disorders. For example, in 
proposed 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'' used in current 114.00D3b(i).

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

    In proposed 114.00A1b, we incorporate the first sentence in the 
last paragraph of current 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 
propose to revise the sentence by adding the phrase ``or their 
treatment.'' We also propose to remove the phrase ``attainment of age-
appropriate skills'' because it is redundant of ``development.''
    Proposed 114.00A2 is essentially the same as proposed 14.00A2 and 
similar to the first and second paragraphs of current 114.00B. We 
propose to expand and clarify 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 also remove the last sentences from 
both the first and second paragraphs of current 114.00B because they 
cross-refer to 14.00 in the part A listings. In part B of these 
proposed rules, we are repeating criteria from part A when they are 
appropriate for evaluating children in part B of the listings so it 
should rarely be necessary to refer back to 14.00 in part A.

Proposed 114.00D--What are the listed autoimmune disorders in these 
listings?

    Proposed 114.00D parallels the structure and content of proposed 
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 proposed 114.00D2, Systemic vasculitis (114.03), as in current 
114.00C3, we provide guidance (in 114.00D2a(ii)) on how we evaluate 
Kawasaki disease and add guidance about anaphylactoid purpura (Henoch-
Schoenlein purpura). Also, in proposed 114.00D2a(ii), we do not use the 
example of giant cell arteritis (temporal arteritis) that is in 
proposed 14.00D2a(ii) because this disorder occurs almost exclusively 
in individuals over 50 years of age.
    In proposed 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 proposed 114.00D3c(i), 
we explain that the extent of involvement and the location of 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 
(101.00) listings. In proposed 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 the special senses listings (102.00) or 
mental disorders listings (112.00). In 114.00D3c(iii) we describe 
features of chronic variants of these syndromes and explain that it is 
appropriate to evaluate the limitations from these disorders under the 
affected body system, such as the musculoskeletal listings (101.00) or 
respiratory system listings (103.00).
    In proposed 114.00D4, Polymyositis and dermatomyositis (114.05), we 
note (in 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

[[Page 44446]]

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 (113.00ff) in proposed 
114.00D4, as we do for adults in proposed 14.00D4. However, unlike in 
the adult rules, we include a reference to calcinosis for children 
because some children develop calcinosis late in the disease. Also, 
when dermatomyositis involves other organs or body systems, we evaluate 
the involvement under the affected body system. In proposed 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 proposed 14.00D4b because MRI findings are not 
considered diagnostic of dermatomyositis in adults. In proposed 
114.00D4c(i), we explain how to evaluate polymyositis and 
dermatomyositis under the listings in newborn and younger infants.
    In proposed 114.00D5, Undifferentiated and mixed connective tissue 
disease (114.06), we note (in proposed 114.00D5a, General) that the 
most common pattern of undifferentiated autoimmune disorders in 
children is mixed connective tissue disease (MCTD). In proposed 
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 proposed 114.00D6, Inflammatory arthritis (114.09), we discuss 
inflammatory arthritides. In proposed 114.00D6a, General, we 
incorporate guidance in current 114.00C2 and 114.00E. We explain that 
we evaluate growth impairment resulting from inflammatory arthritides 
under the criteria in 100.00ff. In proposed 114.00D6b, Inflammatory 
arthritides involving the axial spine (spondyloarthropathies), we 
incorporate the second sentence in current 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.
    Current 114.00E6 provides 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 
explains 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 current 114.00E6 in these proposed 
rules because we believe we no longer need it in the introductory text 
of the listings.
    We added current 114.00E6 in 2002 (66 FR 58010, 58022 and 58045 
(2001)). 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 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 
proposed 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.

Proposed 114.00F--How do we evaluate human immunodeficiency virus (HIV) 
infection?

    Proposed 114.00F parallels the structure and content of proposed 
14.00F in the adult rules, except where the features commonly 
associated with HIV infection differ in children from adults.
    Proposed 114.00F1a, Documentation of HIV infection by definitive 
diagnosis, corresponds to 114.00D3a in the current rules and 14.00F1a 
in the proposed rules. In this section, we propose to lower the age for 
using HIV antibody tests from 24 months of age or older that is in 
current 114.00D3a(i) to 18 months or older in proposed 114.00F1a(i) 
because current clinical practice now accepts these tests beginning at 
18 months of age.
    In proposed 114.00F1a(iv), we clarify the provision in current 
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.
    Proposed 114.00F1b, Documentation of HIV infection in children from 
birth to the attainment of 18 months is new and corresponds to the 
second paragraph in current 114.00D3b, Other acceptable documentation 
of HIV infection in children. However, we are proposing to move this 
information under proposed 114.00F1b to provide documentation of HIV 
infection by definitive diagnosis in children from birth to the 
attainment of 18 months of age who have tested positive for HIV 
antibodies. We also propose to lower the age for children testing 
positive for HIV antibodies from 24 months of age that is in the second 
paragraph of current 114.00D3b to 18 months in proposed 114.00F1b. We 
are proposing to make these changes because current clinical practice 
now accepts these positive test results as diagnostic of HIV infection 
in children beginning at 18 months of age who have tested positive for 
HIV antibodies.
    In proposed 114.00F1b(i), we propose to add ``One or more of the 
tests listed in F1a(ii)-F1a(vii)'' of proposed 114.00F1a because these 
tests are accepted as diagnostic of HIV infection.
    In proposed 114.00F1b(iii), we propose to 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 proposed 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.
    Proposed 114.00F1c, Other acceptable documentation of HIV 
infection, corresponds to current 114.00D3b and proposed 14.00F1b. We 
propose to remove the first paragraph in current 114.00D3b because all 
infants who have HIV antibodies are now tested to determine 
definitively whether they have HIV infection. This makes the first 
paragraph in current 114.00D3b unnecessary.
    In proposed 114.00F2, CD4 tests, we add more detailed guidance to 
the second paragraph of current 114.00D4a by specifying that the extent 
of immune depression correlates with the level of CD4 counts in 
children at 6 years of age or older, the age at which CD4 levels become 
comparable to adult CD4 levels.

[[Page 44447]]

    In proposed 114.00F3b, Other acceptable documentation of the 
manifestations of HIV infection, we explain in proposed 114.00F3b(i) 
for PCP and in 114.00F3b(ii) for CMV that a CD4 count below 200 in 
children 6 years of age or older is supportive evidence of a 
presumptive diagnosis of these manifestations.
    Proposed 114.00F4, HIV manifestations specific to children, 
corresponds to current 114.00D5, HIV in children. In proposed 
114.00F4a, General, we propose to remove the second sentence in current 
114.00D5. That sentence explains that survival times are shorter for 
children who are infected in the first year of life than they are for 
older children and adults. However, due to advances in medical 
treatment this is no longer the case. The second sentence of proposed 
114.00F4a is based on the first paragraph in current 114.00D5.
    In proposed 114.00F4b, Neurologic abnormalities, we make some 
nonsubstantive editorial changes to the second paragraph in current 
114.00D5 in which we explain 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 
proposed 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 proposed 114.00F4c, Bacterial infections, we incorporate the 
last two paragraphs in current 114.00D5. We propose only nonsubstantive 
editorial changes, including removing text that only repeats criteria 
from the listings.

Proposed 114.00G--How will we consider the effect of treatment in 
evaluating your autoimmune disorder, immune deficiency disorder, or HIV 
infection?

    In proposed 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 proposed 14.00G2 for an adult 
because otitis media is more common in children.
    In proposed 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 for children instead of osteoporosis as 
we do in proposed 14.00G3 for adults because impaired growth and 
osteopenia are more common in children.

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

    As in the adult rules, we propose to add listings based on 
functional criteria to each of the listings in the immune system in 
addition to listing 114.08. Current listing 114.08O is the childhood 
listing that corresponds to current adult listing 14.08N, and we are 
proposing to use essentially the same criteria in the other listings as 
we do in this listing. (In the proposed rules, current listing 114.08O 
would become listing 114.08L.) Proposed 114.00I--How do we use the 
functional criteria in these listings?--corresponds to current 114.00D8 
and provides guidance for applying the listings based on functional 
criteria. We propose to revise the current language to reflect the fact 
that there would now be functional listings for each of the listed 
impairments in this body system and for consistency with adult rules 
where appropriate.

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

    In proposed 114.00J2, we repeat the guidance in proposed 14.00J but 
with appropriate references to listings in part B, and we include 
growth impairment under 100.00ff as an example.

How are we proposing to change the criteria in the listings for 
evaluating immune system impairments in children?

Proposed 114.01 Category of Impairments, Immune System Disorders

    As in the adult listings in part A, we propose to remove all 
reference listings from part B. We also propose to add listings like 
114.08O to each of the other listings in this body system. The new 
listings would be proposed listings 114.02B, 114.03B, 114.04D, 114.05E, 
114.06B, 114.07C, 114.09D, and 114.10B. In addition, current listing 
114.08O would be redesignated as listing 114.08L because of the 
deletion of reference listings. The functional criteria in the new 
proposed listings for children would be the same as in current listing 
114.08O (proposed listing 114.08L). They are different from the 
functional criteria in part A because the functional criteria for 
adults are not applicable to the evaluation of functioning in children. 
The childhood functional criteria are the same as in current listing 
114.08O (proposed listing 114.08L); they use the functional criteria in 
listings 112.02 and 112.12.
    The following is a description of the significant proposed changes 
in part B when they are different from the changes we propose in part A 
or require additional explanation.
Proposed Listing 114.04--Systemic Sclerosis (Scleroderma)
    Proposed listings 114.04B1 and 114.04B2 correspond to current 
listing 114.04B1. We propose to change the requirement in current 
listing 114.04B1 for fixed deformity of ``both feet'' to ``one or both 
feet'' and to add ``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 also 
propose to add the criterion of ``toe contractures'' to proposed 
114.04B1 even though toe contractures of listing-level severity would 
be rare in children to make it consistent with the criteria in proposed 
14.04B1. We are retaining the requirement for involvement of both hands 
in proposed listing 114.04B2, because inability to use fine and gross 
movements effectively can only occur when both upper extremities are 
affected. We propose to add the criterion of ``finger contractures'' to 
proposed 114.004B2 for the same reason we are proposing to add ``toe 
contractures'' to proposed 114.04B1.
    Proposed listings 114.04B3 and 114.04B4 correspond to current 
listing 114.04B2, the listing for ``[m]arked destruction or marked 
atrophy of an extremity.'' We propose to revise the rules to
     Remove the word ``marked,''
     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 use 
the upper extremities to perform fine and gross movements effectively.
    We propose to remove 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 propose to replace 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 propose to require 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 propose to 
require ``inability to ambulate effectively'' or ``inability to

[[Page 44448]]

perform fine or gross movements effectively'' to establish an 
impairment that is of listing-level severity, consistent with other 
existing and proposed listings.
    Proposed listing 114.04C, Raynaud's phenomenon, is a new childhood 
listing and has the same criteria as in proposed listing 14.04C for 
adults. Even though listing-level severity would be rare in children 
with Raynaud's phenomenon, it can occur.
Proposed Listing 114.05--Polymyositis and Dermatomyositis
    We propose to remove current 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 proposed listing 114.05E, medically equals the criteria in another 
listing, such as proposed listing 114.05A, or functionally equals the 
listings.
    In proposed listing 114.05D, we propose to revise current listing 
114.05B2 by replacing ``cutaneous calcification'' with ``calcinosis.'' 
We are proposing 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 proposing to 
replace ``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.
Proposed Listing 114.07--Immune deficiency disorders, excluding HIV 
infection
    We propose to remove current listing 114.07B because of advances in 
medical knowledge that now allow us to identify different subgroups of 
thymic dysplastic syndromes. The subgroups of these disorders vary in 
severity, and therefore, they should be evaluated under proposed 
listing 114.07A, B, or C, as appropriate to the particular immune 
deficiency disorder and its effects.
Proposed Listing 114.08--Human Immunodeficiency Virus (HIV) Infection
    In proposed listing 114.08A5, we incorporate current listing 
114.08A6 except to remove ``Other'' as a descriptor to make it 
consistent with the proposed adult listing. We propose to replace 
``acquisition'' as used in current listing 114.08H1 with ``onset'' in 
proposed 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 proposed 
removal of reference listings.
Proposed Listing 114.10-- Sj[ouml]gren's Syndrome
    We propose to add a new listing 114.10 to evaluate Sj[ouml]gren's 
syndrome in children for the same reasons we propose to add a 
Sj[ouml]gren's syndrome listing for adults in part A.

Other Changes

    We propose to make minor conforming changes in current 1.00B and 
101.00B, and 1.00L and 101.00L to reflect changes in the proposed 
immune body system listings.
    We also propose to make minor conforming changes in current 8.00D3 
and 108.00D3 of the skin disorders listings. We would revise these 
sections to indicate that we evaluate Sj[ouml]gren's syndrome under the 
new listing for that disorder, listings 14.10 and 114.10.

Clarity of These Proposed Rules

    Executive Order 12866, as amended by Executive Order 13258, 
requires each agency to write all rules in plain language. In addition 
to your substantive comments on these proposed rules, we invite your 
comments on how to make these proposed rules easier to understand.
    For example:
     Have we organized the material to suit your needs?
     Are the requirements in the rules clearly stated?
     Do the rules contain technical language or jargon that is 
not clear?
     Would a different format (grouping and order of sections, 
use of headings, paragraphing) make the rules easier to understand?
     Would more (but shorter) sections be better?
     Could we improve clarity by adding tables, lists, or 
diagrams?
     What else could we do to make the rules easier to 
understand?

Regulatory Procedures

Executive Order 12866

    We have consulted with the Office of Management and Budget (OMB) 
and determined that these proposed rules meet the requirements for a 
significant regulatory action under Executive Order 12866, as amended 
by Executive Order 13258. Thus, they were subject to OMB review.

Regulatory Flexibility Act

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

Paperwork Reduction Act

    These proposed rules contain reporting requirements at 14.00B, 
14.00D, 14.00E, 14.00F, 114.00B, 114.00D, 114.00E, 114.00F, 114.08 and 
114.09. The public reporting burden is accounted for in the Information 
Collection Requests for the various forms that the public uses to 
submit the information to SSA. Consequently, a 1-hour placeholder 
burden is being assigned to the specific reporting requirement(s) 
contained in these rules. We are seeking clearance of the burdens 
referenced in these rules because they were not considered during the 
clearance of the forms. An Information Collection Request has been 
submitted to OMB. We are soliciting comments on the burden estimate; 
the need for the information; its practical utility; ways to enhance 
its quality, utility and clarity; and on ways to minimize the burden on 
respondents, including the use of automated collection techniques or 
other forms of information technology. Comments should be submitted 
and/or faxed to the Office of Management and Budget and to the Social 
Security Administration at the following addresses/numbers:

Office of Management and Budget, Attn: Desk Officer for SSA, New 
Executive Office Building, Room 10230, 725 17th St., NW., Washington, 
DC 20530. Fax Number: 202-395-6974.
Social Security Administration, Attn: SSA Reports Clearance Officer, 
Rm. 1338 Annex Building, 6401 Security Boulevard, Baltimore, MD 21235-
6401. Fax Number: 410-965-6400.

    Comments can be received for up to 60 days after publication of 
this notice, and your comments will be most useful if received by SSA 
within 30 days of publication. To receive a copy of the OMB clearance 
package, you may call the SSA Reports Clearance Officer on 410-965-
0454.

References

    We consulted the following sources when developing these proposed 
rules:

Bartlett, J.G. and Gallant, J.E., Medical Management of HIV 
Infection (Johns Hopkins University 2003).

[[Page 44449]]

Burchett, S.A. and Pizzo, P.A., HIV Infection in Infants, Children, 
and Adolescents, Pediatrics in Review, 24(6), 186-194 (2001).
Davidson, A. and Diamond, B., Autoimmune Diseases, The New England 
Journal of Medicine, 345(5), 1-21 (2001).
Furst, D.E., Stem Cell Transplantation for Autoimmune Disease: 
Progress and Problems, Current Opinion in Rheumatology, 14(3), 220-
224 (2002).
Harris, E.D., et al., Eds., Kelley's Textbook of Rheumatology, 
(Elsevier, 7th ed. 2005).
Klippel, J.H., et al., Eds., Primer on the Rheumatic Diseases, 
(Arthritis Foundation, 12th ed. 2001).
Sicherer, S.H., et al., Primary Immunodeficiency Diseases in Adults, 
Journal of American Medical Association, 279:58 (1998).
Tyndall, A. and Koike, T., High-dose immunoablative therapy with 
hematopoietic stem cell support in the treatment of severe 
autoimmune disease: current status and future direction, Internal 
Medicine, 41(8), 608-12 (2002).

    These references are included in the rulemaking record for these 
proposed rules and are available for inspection by interested persons 
by making arrangements with the contact person shown in this preamble.

(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 20 CFR Part 404

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

    Dated: July 28, 2006.
Jo Anne B. Barnhart,
Commissioner of Social Security.

    For the reasons set out in the preamble, we propose to amend 
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- )

    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), Public Law 104-193, 
110 Stat. 2105, 2189.

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

    2. Appendix 1 to subpart P of part 404 is amended as follows:
    a. Revise the expiration date in item 15 of the introductory 
text before part A of appendix 1.
    b. Revise the second sentence of section 1.00B1 of part A of 
appendix 1.
    c. Revise the fourth sentence of section 1.00L of part A of 
appendix 1.
    d. Revise section 8.00D3 of part A of appendix 1.
    e. Revise section 14.00 of part A of appendix 1.
    f. Revise the second sentence of section 101.00B1 of part B of 
appendix 1.
    g. Revise the fourth sentence of section 101.00L of part B of 
appendix 1.
    h. Revise section 108.00D3 of part B of appendix 1.
    i. Revise section 114.00 of part B of appendix 1.

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

* * * * *
    15. Immune System Disorders (14.00 and 114.00): (date 8 years 
from the effective date of the final rules.)
* * * * *
Part A
* * * * *
1.00 Musculoskeletal System
* * * * *
    B. Loss of function.
    1. General. * * * For inflammatory arthritides that may result 
in loss of function because of inflammatory peripheral joint or 
axial arthritis or sequelae, or because of extra-articular features, 
see 14.00D6. * * *
* * * * *
    L. Abnormal curvatures of the spine. * * * 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. How do we assess impairments that may affect the skin and 
other body systems?
* * * * *
    3. Autoimmune disorders and other immune system disorders (for 
example, systemic lupus erythematosus, 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 listings in 14.00. We evaluate 
lupus erythematosus under 14.02, scleroderma under 14.04, 
symptomatic HIV infection under 14.08, and Sj[ouml]gren's syndrome 
under 14.10.
* * * * *
14.00 Immune System Disorders

    A. What disorders do we evaluate under the immune system 
listings?
    1. We evaluate immune system disorders that cause dysfunction in 
one or more components of your immune system.
    a. These listings are examples of immune system disorders that 
are severe enough to prevent you from doing any gainful activity. 
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 fatigue, fever, malaise, diffuse 
musculoskeletal pain, or involuntary weight loss, can also result in 
extreme limitation.
    c. In this preface, 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 is caused by a specific retrovirus and 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? Generally, we need your medical history, report(s) 
of physical examination, 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 one that is 
generally accepted and consistent with the

[[Page 44450]]

prevailing state of medical knowledge and clinical practice to 
support the evaluation and diagnosis of the impairment.
    2. Constitutional symptoms or signs means 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, 
the effect is in 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 describes 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. What are the listed autoimmune disorders in these listings?
    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 (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 (inflammatory arthritis) disorders. 
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. Usually the results will be in your medical 
records.
    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. The extent of involvement of 
linear scleroderma and a description of the lesions are important in 
assessing the severity of the impairment. 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 
contracture(s) and leg length discrepancies. In such cases, 
evaluation under the musculoskeletal (1.00ff) listing may be 
appropriate.
    (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 special senses listings (2.00ff) or mental disorders 
listings (12.00ff).
    (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 (1.00ff) 
or respiratory system listings (3.00ff).
    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. Symmetric weakness, and less 
frequently pain and tenderness of the proximal limb-girdle (shoulder 
or pelvic) musculature, are the most common manifestations. There 
may also be involvement of the cervical, cricopharyngeal, 
esophageal, intercostal, and diaphragmatic muscles.
    b. Documentation of polymyositis and dermatomyositis. Generally, 
but not always, polymyositis is associated with elevated serum 
muscle enzymes (creatine phosphokinase (CPK), aminotransferases, 
aldolase), and characteristic abnormalities on electromyography and 
muscle biopsy. In dermatomyositis there are characteristic skin 
findings in addition to the findings of polymyositis.
    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.

[[Page 44451]]

    (ii) We use the malignant neoplastic diseases listings (13.00ff) 
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 systemic 
lupus erythematosus 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 inflammatory arthritides include 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 functional 
limitations in ambulation or performance of fine and gross 
movements. However, in combination with extra-articular features, 
including constitutional symptoms or signs (fatigue, fever, malaise, 
involuntary weight loss), inflammatory arthritis may result in an 
extreme limitation.
    b. Inflammatory arthritides involving the axial spine 
(spondyloarthropathies). In adults, inflammatory arthritides 
involving the axial spine may be associated with heterogeneous 
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 arthritides involving the peripheral joints. The 
inflammatory arthropathies 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 arthritides. Generally, but not 
always, the diagnosis of inflammatory arthritis is made by the 
clinical features and serologic findings described in the most 
recent edition of the Primer on Rheumatic Diseases published by the 
Arthritis Foundation.
    e. How we evaluate the inflammatory arthritides 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 two or more major peripheral joints resulting in the 
inability to ambulate effectively or inability to perform fine and 
gross movements effectively, as defined in 14.00C6 and 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 when the arthritis does not result in the extreme limitation 
in 14.09A or 14.09C1, involves one or more major peripheral joints, 
or involves other joints, but is complicated by extra-articular 
features that cumulatively result in an ``extreme'' (very serious) 
limitation or ``marked'' (serious) limitations in at least two areas 
of functioning. Extra-articular impairments may also meet listings 
in other body systems.
    (iii) Extra-articular features of inflammatory arthritis may 
involve any body system. Commonly occurring extra-articular 
impairments include: 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), and immune 
system (Felty's syndrome (hypersplenism with compromised immune 
competence)) disorders.
    (iv) If permanent deformity of a major peripheral joint is the 
dominant feature of your impairment, we evaluate your impairment 
under 1.02.
    (v) If there has been surgical reconstruction of a major weight-
bearing joint, we evaluate your impairment under 1.03.
    (vi) 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). 
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 evaluate immune deficiency disorders, excluding HIV 
infection (14.07)?
    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

[[Page 44452]]

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 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.
    a. Documentation of HIV infection by definitive diagnosis. 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 if you have an opportunistic 
disease that is predictive of a defect in cell-mediated immunity 
(for example, toxoplasmosis of the brain, Pneumocystis carinii 
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 200/mme or less (14 percent or less) 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 by other generally acceptable methods consistent with 
the prevailing state of medical knowledge and clinical practice. 
When you have had laboratory testing for a manifestation of HIV 
infection, we will make every reasonable effort to obtain reports of 
the results of that testing.
    a. Documentation of the manifestations of HIV infection by 
definitive diagnosis. 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. If no definitive 
evidence is available, we may document the manifestations of HIV 
infection with other appropriate evidence. 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.
    (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. (Note: Pneumocystis carinii is now known as 
Pneumocystis jiroveci; however, ``PCP'' remains in common usage for 
the pneumonia caused by this organism.) Supportive evidence 
includes: Fever, dyspnea, hypoxia, and CD4 count below 200. Also 
supportive are bilateral lung interstitial infiltrates on x-ray, or 
a typical pattern on CT scan, or a gallium scan positive for 
pulmonary uptake. Response to anti-PCP therapy usually requires 5-7 
days.
    (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 on 
funduscopic exam) 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 identifies a history of infection with CMV, but it 
does not confirm an active disease process. Therefore, a presumptive 
diagnosis of CMV disease requires corroborating evidence that CMV is 
causing the disease. Supportive evidence includes: Fever, positive 
CMV serology test, 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 
made by 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.
    4. 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

[[Page 44453]]

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. As used in 14.08H, ``significant 
involuntary weight loss'' does not correspond to a specific minimum 
amount or percentage of weight loss. For purposes of this listing, 
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.)
    G. How will we consider the effect 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 
both 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 regimes, 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 these 
disorders 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 function 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 on a sustained 
basis. We will also consider whether you have chronic side effects 
from these or other medications, including 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, glucose 
intolerance, and lactic acidosis. In addition, medications used in 
the treatment of HIV infection may also have effects on mental 
function, including cognition (for example, memory), concentration, 
and mood, and may result in malaise, 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 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 or 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 impairment 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 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 
listings, but your immune system impairment 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 
constitutional symptoms or pain?
    Your symptoms, including pain, 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.
    I. How do we use the functional criteria in these listings?
    1. The following listings in this body system include standards 
for evaluating the

[[Page 44454]]

limitations resulting from repeated manifestations of immune system 
disorders that do not meet the criteria of the other sections of 
their respective listings: 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 arthritides; and 14.10B, 
for Sj[ouml]ogren'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(s) 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.
    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, 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 ``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, fatigue, anxiety, or difficulty 
concentrating, imposed 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 ``marked'' difficulty maintaining 
social functioning if you have serious limitation in social 
interaction on a sustained basis because of symptoms, such as pain, 
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 ``marked'' difficulty completing tasks if you have 
serious limitation in your ability to sustain concentration or pace 
adequate to complete work-related tasks because of symptoms, such as 
pain, 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 1.00ff.
    b. Ocular involvement, such as dry eye, under 2.00ff.
    c. Respiratory impairments, such as pleuritis, under 3.00ff.
    d. Cardiovascular impairments, such as cardiomyopathy, under 
4.00ff.
    e. Digestive impairments, such as hepatitis (including hepatitis 
C), under 5.00ff.
    f. Genitourinary impairments, such as nephropathy, under 6.00ff.
    g. Hematologic abnormalities, such as anemia, granulocytopenia, 
and thrombocytopenia, under 7.00ff.
    h. Skin impairments, such as persistent fungal and other 
infectious skin eruptions, and photosensitivity, under 8.00ff.
    i. Neurologic impairments, such as neuropathy or seizures, under 
11.00ff.
    j. Mental disorders, such as depression, anxiety, or cognitive 
deficits, under 12.00ff.
    k. Allergic disorders, such as asthma or atopic dermatitis, 
under 3.00ff or 8.00ff 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 following constitutional symptoms or 
signs: Severe fatigue, fever, malaise, or involuntary weight loss.

OR

    B. Repeated manifestations of SLE but without the requisite 
findings in A, resulting in at least two of the constitutional 
symptoms or signs in A2, 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 following constitutional symptoms or 
signs: Severe fatigue, fever, malaise, or involuntary weight loss.

OR

    B. Repeated manifestations of systemic vasculitis but without 
the requisite findings

[[Page 44455]]

in A, resulting in at least two of the constitutional symptoms or 
signs in A2, 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 following 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 of a toe or finger in at least two extremities, or 
of a toe and finger; 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) 
but without the requisite findings in A, B, or C, resulting in at 
least two of the constitutional symptoms or signs in A2, 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 
but without the requisite findings in A, B, or C, resulting in at 
least two of the following 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 following constitutional symptoms or 
signs: Severe fatigue, fever, malaise, or involuntary weight loss.

OR

    B. Repeated manifestations of undifferentiated or mixed 
connective tissue disease but without the requisite findings in A, 
resulting in at least two of the constitutional symptoms or signs in 
A2, 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 but 
without the requisite findings in A or B, resulting in at least two 
of the following 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
    4. Multiple or recurrent bacterial infection(s), 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 another 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 carinii (jiroveci) pneumonia or extrapulmonary 
pneumocystis carinii (jiroveci) 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

[[Page 44456]]

    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 anus.

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 (or other significant 
involuntary weight loss, as described in 14.00F5) and, in the 
absence of a concurrent illness that could explain the findings, 
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 impairment) resulting in significant, documented symptoms or 
signs (for example, fatigue, fever, malaise, involuntary weight 
loss, pain, night sweats, nausea, vomiting, headaches, or insomnia) 
and one of the following at the marked level (as defined in 
14.00I5):
    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 deformity in two or more major 
peripheral joints resulting in the inability to ambulate effectively 
or inability to perform fine and gross movements effectively as 
defined in 14.00C6 and 14.00C7.

OR

    B. Inflammation or deformity in one or more major peripheral 
joints, but with less joint involvement than in A and extra-
articular features that do not satisfy the criteria of a listing, 
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 following 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 spines 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 but without 
the requisite findings in A, B, or C, resulting in at least two of 
the constitutional symptoms or signs in B2, 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 Sjogren'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 following constitutional symptoms or 
signs: Severe fatigue, fever, malaise, or involuntary weight loss.

OR

    B. Repeated manifestations of Sjogren's syndrome but without the 
requisite findings in A, resulting in at least two of the 
constitutional symptoms or signs in A2, 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
* * * * *
101.00 Musculoskeletal System
* * * * *
    B. Loss of function.
    1. General. * * * For inflammatory arthritides that result in 
loss of function because of inflammatory peripheral joint or axial 
arthritis or sequelae, or because of extra-articular features, see 
114.00D6. * * *
* * * * *
    L. Abnormal curvatures of the spine. * * * 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 114.09C. * * *
* * * * *
108.00 Skin Disorders
* * * * *
    D. How do we assess impairments that may affect the skin and 
other body systems?
* * * * *
    3. Autoimmune disorders and other immune system disorders (for 
example, systemic lupus erythematosus, 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 listings in 114.00. We evaluate 
lupus erythematosus under 114.02, scleroderma under 114.04, 
symptomatic HIV infection under 114.08, and Sjogren's syndrome under 
114.10.
* * * * *
114.00 Immune System Disorders
    A. What disorders do we evaluate under the immune system 
listings?
    1. We evaluate immune system disorders that cause dysfunction in 
one or more components of your immune system.
    a. These listings are examples of immune system disorders that 
are severe enough to result in marked and severe functional 
limitations. 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 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 performance of 
age-appropriate activities.

[[Page 44457]]

    c. In this preface, 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 is caused by a specific retrovirus and 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, report(s) 
of physical examination, 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 one that is 
generally accepted and consistent with the prevailing state of 
medical knowledge and clinical practice to support the evaluation 
and diagnosis of the impairment.
    2. Constitutional symptoms or signs means 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, 
the effect is in 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 describes 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. What are the listed autoimmune disorders in these listings?
    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 (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 (inflammatory arthritis) disorders. 
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 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 (104.00ff). 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 (105.00ff) or genitourinary (106.00ff) 
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. Usually the results will be in your medical 
records.
    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. The extent of 
involvement of linear scleroderma and a description of the lesions 
are important in assessing the severity of the impairment.

[[Page 44458]]

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 contracture(s) and leg length 
discrepancies. In such cases, evaluation under the musculoskeletal 
(101.00ff) listings may be appropriate.
    (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 special senses listings (102.00ff) or mental 
disorders listings (112.00ff).
    (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 impairment and may also lead to 
restrictive pulmonary disease. We evaluate these variants of the 
disease under the criteria in the musculoskeletal listings 
(101.00ff) or respiratory system listings (103.00ff).
    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. 
Symmetric weakness, and less frequently pain and tenderness of the 
proximal limb-girdle (shoulder or pelvic) musculature, are the most 
common manifestations. 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 rash. 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, 
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 rash, and elevated 
serum muscle enzymes. Additional evidence of the diagnosis of 
childhood dermatomyositis is depiction on MRI of muscle inflammation 
or vasculitis.
    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 the child's 
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 systemic 
lupus erythematosus 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 inflammatory arthritides include 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 functional 
limitations in ambulation or performance of fine and gross 
movements. However, in combination with extra-articular features, 
including constitutional symptoms or signs (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 arthritides 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 100.00ff.
    b. Inflammatory arthritides involving the axial spine 
(spondyloarthropathies). In children, inflammatory arthritides 
involving the axial spine may be associated with heterogeneous 
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 arthritides involving the peripheral joints. In 
children, the inflammatory arthropathies involving peripheral joints 
may be associated with disorders such as:
    (i) Juvenile 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 arthritides. Generally, but not 
always, the diagnosis of inflammatory arthritis is made by the 
clinical features and serologic findings described in the most 
recent edition of the Primer on Rheumatic Diseases published by the 
Arthritis Foundation.
    e. How we evaluate the inflammatory arthritides 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 two or more major peripheral joints 
resulting in the inability to ambulate effectively or inability to 
perform fine and gross movements effectively, as defined in 114.00C6 
and 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 when the arthritis does not result in the extreme limitation 
in 114.09A or 114.09C1, involves one or more major peripheral 
joints, or involves other joints, but is complicated by extra-
articular features that cumulatively result in an ``extreme'' (very 
serious) limitation or ``marked'' (serious) limitations in at least 
two areas of functioning. Extra-articular impairments may also meet 
listings in other body systems.
    (iii) Extra-articular features of inflammatory arthritis may 
involve any body system. Commonly occurring extra-articular 
impairments include: Musculoskeletal (heel enthesopathy), 
ophthalmologic (iridocyclitis, keratoconjunctivitis sicca, uveitis), 
pulmonary (pleuritis, pulmonary fibrosis or

[[Page 44459]]

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), and immune system (Felty's syndrome (hypersplenism with 
compromised immune competence)) disorders.
    (iv) If permanent deformity of a major peripheral joint is the 
dominant feature of your impairment, we evaluate your impairment 
under 101.02.
    (v) If there has been surgical reconstruction of a major weight-
bearing joint, we evaluate your impairment under 101.03.
    (vi) 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). 
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 evaluate immune deficiency disorders, excluding HIV 
infection (114.07)?
    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 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 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.
    a. Documentation of HIV infection by definitive diagnosis. 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, 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. 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/mm3 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/mm3 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 if you have an opportunistic 
disease that is predictive of a defect in cell-mediated immunity 
(for example, Pneumocystis carinii 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

[[Page 44460]]

of their T-helper lymphocytes (CD4 cells). The extent of immune 
suppression correlates with the level or rate of decline of the CD4 
count. At age 6, children begin to have CD4 counts comparable to the 
levels found in adults. Generally, in these children when the CD4 
count is 200/mm3 or less (14 percent or less) 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 by other generally acceptable methods consistent with 
the prevailing state of medical knowledge and clinical practice. 
When you have had laboratory testing for a manifestation of HIV 
infection, we will make every reasonable effort to obtain reports of 
the results of that testing.
    a. Documentation of the manifestations of HIV infection by 
definitive diagnosis. 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. If no definitive 
evidence is available, we may document the manifestations of HIV 
infection with other appropriate evidence. 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.
    (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. (Note: Pneumocystis carinii is now known as 
Pneumocystis jiroveci; however, ``PCP'' remains in common usage for 
the pneumonia caused by this organism.) Supportive evidence 
includes: Fever, dyspnea, hypoxia, and CD4 count below 200 in 
children 6 years of age or older. Also supportive are bilateral lung 
interstitial infiltrates on x-ray, or a typical pattern on CT scan, 
or a gallium scan positive for pulmonary uptake. Response to anti-
PCP therapy usually requires 5-7 days.
    (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 on 
funduscopic exam) 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 identifies a history of infection with CMV, but it 
does not confirm an active disease process. Therefore, a presumptive 
diagnosis of CMV disease requires corroborating evidence that CMV is 
causing the disease. Supportive evidence includes: Fever, positive 
CMV serology test, 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 
made by 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.
    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 6 
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.
    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 
CT scan or MRI. Neurologic abnormalities in infants and young 
children may present as serious developmental delays or in the loss 
of previously acquired developmental milestones. In school-age 
children and adolescents, this type of neurologic abnormality would 
generally present as the loss of previously acquired intellectual 
abilities. This may be evidenced by a decrease in intelligence 
quotient (IQ) scores, by a child forgetting information he or she 
previously learned, by being unable 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 will we consider the effect 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 
both 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 individuals with immune system disorders receive 
treatment both for their immune system disorders and for the 
manifestations of the disorders or co-occuring 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,

[[Page 44461]]

requirements for changes in therapeutic regimes, 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 these disorders 
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 function 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 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, glucose 
intolerance, and lactic acidosis. In addition, medications used in 
the treatment of HIV infection may also have effects on mental 
function, including cognition (for example, memory), concentration, 
and mood, and may result in malaise, 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 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 or 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 impairment 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 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 
listings, but your immune system impairment may medically equal a 
listing or functionally equal the listings.
    H. How do we consider your symptoms, including your 
constitutional symptoms or pain?
    Your symptoms, including pain, 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, and 
416.929.
    I. How do we use the functional criteria in these listings?
    1. The following listings in this body system include standards 
for evaluating the limitations resulting from manifestations of 
immune system disorders that do not meet the criteria of the other 
sections of their respective listings: 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 
arthritides; and 114.10B, for Sj[ouml]gren'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(s) 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. To satisfy the functional criterion in a listing, your immune 
system disorder must result in an ``extreme'' limitation in one 
domain of functioning or ``marked'' limitations 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, 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 imitations. 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 100.00ff.
    b. Musculoskeletal involvement, such as surgical reconstruction 
of a joint, under 101.00ff.
    c. Ocular involvement, such as dry eye, under 102.00ff
    d. Respiratory impairments, such as pleuritis, under 103.00ff.
    e. Cardiovascular impairments, such as cardiomyopathy, under 
104.00ff.
    f. Digestive impairments, such as hepatitis (including hepatitis 
C), under 105.00ff.

[[Page 44462]]

    g. Genitourinary impairments, such as nephropathy, under 
106.00ff.
    h. Hematologic abnormalities, such as anemia, granulocytopenia, 
and thrombocytopenia, under 107.00ff.
    i. Skin impairments, such as persistent fungal and other 
infectious skin eruptions, and photosensitivity, under 108.00ff.
    j. Neurologic impairments, such as neuropathy or seizures, under 
111.00ff.
    k. Mental disorders, such as depression, anxiety, or cognitive 
deficits, under 112.00ff.
    l. Allergic disorders, such as asthma or atopic dermatitis, 
under 103.00ff or 108.00ff 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 following 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 following 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 following 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 of a toe or finger in at least two extremities, or 
of a toe and finger; 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:
    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) and 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 following 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 manifestations(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

[[Page 44463]]

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 infection(s) (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; or
    5. Multiple or recurrent bacterial infection(s), 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 another 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 carinii (jiroveci) pneumonia or extrapulmonary 
pneumocystis carinii (jiroveci) 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 anus.

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 
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

    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 deformity in two or more major 
peripheral joints resulting in the inability to ambulate effectively 
or the inability to perform fine and gross movements effectively as 
defined in 114.00C6 and 114.00C7.

OR

    B. Inflammation or deformity in one or more major peripheral 
joints, but with less joint involvement than in A and extra-
articular features that do not satisfy the criteria of a listing, 
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 following 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 spines 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

[[Page 44464]]

    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 n 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 following 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. 06-6655 Filed 8-3-06; 8:45 am]
BILLING CODE 4191-02-P