Revised Medical Criteria for Evaluating Immune System Disorders, 14570-14616 [E8-5023]

Agencies

• Social Security Administration

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



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Part II





Social Security Administration





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20 CFR Part 404



Revised Medical Criteria for Evaluating Immune System Disorders; Final 
rule

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

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SOCIAL SECURITY ADMINISTRATION

20 CFR Part 404

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


Revised Medical Criteria for Evaluating Immune System Disorders

AGENCY: Social Security Administration.

ACTION: Final Rules.

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

DATES: These rules are effective June 16, 2008.

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

SUPPLEMENTARY INFORMATION: 

Electronic Version

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

Background

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

What Programs Do These Final Rules Affect?

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

Who Can Get Disability Benefits?

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

How do we define disability?

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

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

How do we decide whether you are disabled?

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

What are the listings?

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

[[Page 14571]]

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

How do we use the listings?

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

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

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

Why are we revising the listings for immune system disorders?

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

How did we develop these final rules?

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

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

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

When will we start to use these final rules?

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

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

How long will these final rules be effective?

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

What revisions are we making with these final rules?

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

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

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

[[Page 14573]]

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

14.00 Immune System Disorders

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

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

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

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

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

[[Page 14574]]

Final 14.00C--Definitions

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

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

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

[[Page 14575]]

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

[[Page 14576]]

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

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

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

[[Page 14577]]

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

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

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

[[Page 14578]]

percent'' and clarified that the reference is to the percentage of CD4 
cells to the total lymphocyte count. We made the same changes 
throughout these final rules for consistency with these corrections. We 
also made nonsubstantive editorial changes in this paragraph.
    In final 14.00F3, Documentation of the manifestations of HIV 
infection, we incorporate the information in prior 14.00D4 with 
nonsubstantive editorial changes. Like final 14.00F1 and prior 14.00D4, 
final 14.00F3 is divided into two main parts:
     Final 14.00F3a, Definitive documentation of the 
manifestations of HIV infection, incorporates the first paragraph in 
prior 14.00D4a and explains how we document manifestations of HIV 
infection definitively.
     Final 14.00F3b, Other acceptable documentation of the 
manifestations of HIV infection, incorporates information that was in 
the first paragraph of prior 14.00D4b and explains how we document 
manifestations of HIV infection when we do not have definitive 
evidence.
    We are revising the language of proposed 14.00F3b to clarify our 
original intent. In the prior rule, we indicated that ``if no 
definitive laboratory evidence is available, manifestations of HIV 
infection may be documented by medical history, clinical and laboratory 
findings, and diagnosis(es) indicated in the medical evidence.'' The 
sentence may have implied that we needed to have all of the things 
listed (medical history and clinical findings and laboratory findings 
and diagnosis(es)) to determine that you have a manifestation of HIV 
infection when we do not have definitive laboratory findings. That was 
not our intent, so we are clarifying in the final rule that we may need 
only some of this information to make a finding that you have a 
manifestation of HIV infection, depending on the prevailing state of 
medical knowledge and clinical practice. We are also clarifying what we 
mean by ``laboratory findings'' in this context; that is, laboratory 
findings that do not in themselves definitively establish the existence 
of an HIV-related manifestation. In response to a public comment on the 
NPRM, we are also clarifying in final 14.00F3b that the manifestations 
that are listed are only examples of manifestations that can be 
diagnosed without definitive evidence. We will accept a presumptive 
diagnosis of any manifestation of HIV infection so long as the method 
used to make the diagnosis is consistent with the prevailing state of 
medical knowledge and clinical practice.
    In 14.00D4 of the prior rules we provided specific guidance for 
documenting one particular manifestation of HIV infection without 
definitive evidence: Cytomegalovirus (CMV) disease. In final 14.00F3b, 
we expand the section to include three additional manifestations, 
including a manifestation we added in response to a public comment on 
the NPRM. The revised guidance is as follows:
     In final 14.00F3b(i), we explain that PCP is frequently 
diagnosed presumptively without definitive evidence and provide 
examples of evidence that is supportive of a presumptive diagnosis of 
PCP. Because we removed the word ``carinii'' in a change we made in 
final 14.00F1b, we no longer need the parenthetical note we proposed to 
include in 14.00F3b(i); therefore, we have not included it in these 
final rules. In response to a public comment on the NPRM, we also added 
``no evidence of bacterial pneumonia'' to the list of evidence that is 
supportive of a presumptive diagnosis of PCP. For consistency with a 
change we made in final 14.00F3b(ii) in response to a public comment on 
the NPRM, we also indicate that supportive evidence of a presumptive 
diagnosis of PCP ``may'' include the items we list. This is not a 
change in the meaning of the proposed rule, only a clarification.
     In final 14.00F3b(ii), we incorporate and expand the 
information now in the second paragraph of prior 14.00D4b, regarding 
the documentation of CMV disease. However, in an editorial change from 
the NPRM, we revised the second and fourth sentences and removed the 
third sentence in proposed 14.00F3b(ii). In the NPRM, we stated that a 
serology test ``identifies a history of infection with CMV, but it does 
not confirm an active disease process.'' We revised this to sta