Revised Medical Criteria for Evaluating Immune System Disorders

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.

SUMMARY:

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

DATES:

These rules are effective June 16, 2008.

FOR FURTHER INFORMATION CONTACT:

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

http://www.socialsecurity.gov/

.

SUPPLEMENTARY INFORMATION:

Electronic Version

The electronic file of this document is available on the date of publication in the

Federal Register

at

http://www.gpoaccess.gov/fr/index.html

.

Background

We are revising and making final the rules we proposed for evaluating immune system disorders in the Notice of Proposed Rulemaking (NPRM) published in the

Federal Register

on August 4, 2006 (71 FR 44432, corrected at 71 FR 46983). We provide a summary of the provisions of the final rules below, with an explanation of the changes we have made from the text in the NPRM. We then provide summaries of the public comments on the NPRM and our reasons for adopting or not adopting the recommendations in those comments in the section “Public Comments on the NPRM.” The final rule language follows that section.

What Programs Do These Final Rules Affect?

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

Who Can Get Disability Benefits?

Under title II of the Act, we provide for the payment of disability benefits if you are disabled and belong to one of the following three groups:

• Workers insured under the Act,

• Children of insured workers, and

• Widows, widowers, and surviving divorced spouses (see § 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:

If you file a claim under . . .

And you are . . .

Disability means you have a medically determinable impairment(s) as described above that results in . . .

title II

an adult or a child

the inability to do any substantial gainful activity (SGA).

title XVI

an individual age 18 or older

the inability to do any SGA.

title XVI

an individual under age 18

marked and severe 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 §§ 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 § 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 §§ 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

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 § 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 §§ 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 §§ 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 §§ 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 § 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.

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 Sjo

gren'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?

• 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 §§ 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° (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.

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 Sjo

gren'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

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

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° 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,

Sjögren's syndrome (14.10),

is new. As already noted, we are adding a listing for Sjögren'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 Sjögren's syndrome may be associated with other autoimmune disorders. In final 14.00D7b,

Documentation of Sjögren's syndrome,

we also explain that if you have Sjögren'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 Sjögren'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

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

percent” and clarified that the reference is to the percentage of CD4 cells to the total lymphocyte count. We made the same changes throughout these final rules for consistency with these corrections. We also made nonsubstantive editorial changes in this paragraph.

In final 14.00F3,

Documentation of the manifestations of HIV infection,

we incorporate the information in prior 14.00D4 with nonsubstantive editorial changes. Like final 14.00F1 and prior 14.00D4, final 14.00F3 is divided into two main parts:

• Final 14.00F3a,

Definitive documentation of the manifestations of HIV infection,

incorporates the first paragraph in prior 14.00D4a and explains how we document manifestations of HIV infection definitively.

• Final 14.00F3b,

Other acceptable documentation of the manifestations of HIV infection,

incorporates information that was in the first paragraph of prior 14.00D4b and explains how we document manifestations of HIV infection when we do not have definitive evidence.

We are revising the language of proposed 14.00F3b to clarify our original intent. In the prior rule, we indicated that “if no definitive laboratory evidence is available, manifestations of HIV infection may be documented by medical history, clinical and laboratory findings, and diagnosis(es) indicated in the medical evidence.” The sentence may have implied that we needed to have all of the things listed (medical history and clinical findings and laboratory findings and diagnosis(es)) to determine that you have a manifestation of HIV infection when we do not have definitive laboratory findings. That was not our intent, so we are clarifying in the final rule that we may need only some of this information to make a finding that you have a manifestation of HIV infection, depending on the prevailing state of medical knowledge and clinical practice. We are also clarifying what we mean by “laboratory findings” in this context; that is, laboratory findings that do not in themselves definitively establish the existence of an HIV-related manifestation. In response to a public comment on the NPRM, we are also clarifying in final 14.00F3b that the manifestations that are listed are only examples of manifestations that can be diagnosed without definitive evidence. We will accept a presumptive diagnosis of any manifestation of HIV infection so long as the method used to make the diagnosis is consistent with the prevailing state of medical knowledge and clinical practice.

In 14.00D4 of the prior rules we provided specific guidance for documenting one particular manifestation of HIV infection without definitive evidence:

Cytomegalovirus

(CMV) disease. In final 14.00F3b, we expand the section to include three additional manifestations, including a manifestation we added in response to a public comment on the NPRM. The revised guidance is as follows:

• In final 14.00F3b(i), we explain that PCP is frequently diagnosed presumptively without definitive evidence and provide examples of evidence that is supportive of a presumptive diagnosis of PCP. Because we removed the word “

carinii

” in a change we made in final 14.00F1b, we no longer need the parenthetical note we proposed to include in 14.00F3b(i); therefore, we have not included it in these final rules. In response to a public comment on the NPRM, we also added “no evidence of bacterial pneumonia” to the list of evidence that is supportive of a presumptive diagnosis of PCP. For consistency with a change we made in final 14.00F3b(ii) in response to a public comment on the NPRM, we also indicate that supportive evidence of a presumptive diagnosis of PCP “may” include the items we list. This is not a change in the meaning of the proposed rule, only a clarification.

• In final 14.00F3b(ii), we incorporate and expand the information now in the second paragraph of prior 14.00D4b, regarding the documentation of CMV disease. However, in an editorial change from the NPRM, we revised the second and fourth sentences and removed the third sentence in proposed 14.00F3b(ii). In the NPRM, we stated that a serology test “identifies a history of infection with CMV, but it does not confirm an active disease process.” We revised this to state that a serology test “does not establish a definitive diagnosis of CMV disease, but it does offer supportive evidence of a presumptive diagnosis of CMV disease.” Due to this revision, we removed a positive CMV serology test from the list of examples of clinical findings that are supportive of a presumptive diagnosis of CMV that were in the fourth sentence of the proposed section, and revised the sentence to indicate that the examples provided are other clinical findings that support a presumptive diagnosis of CMV. We removed the third sentence because it was unnecessary. These changes are not substantive, only a clarification of the proposed rules. As in the NPRM, we do not include “documentation of CMV disease requires confirmation by biopsy” as in the last sentence of the second paragraph of prior 14.00D4b because we are providing information on documentation other than definitive laboratory findings. Also, instead of stating that we can use generally acceptable methods to confirm the diagnosis of CMV, we provide examples of evidence, such as fever and a positive CMV serology test, that is supportive of a presumptive diagnosis of CMV disease. In response to a public comment on the NPRM, we are clarifying that an individual need not have all of the findings we list by indicating that supporting evidence “may” include these findings.

• In final 14.00F3b(iii), we explain how toxoplasmosis of the brain is presumptively diagnosed since the definitive method of diagnosing toxoplasmosis of the brain by biopsy is not commonly performed.

• In final 14.00F3b(iv) we provide guidance about how candidiasis of the esophagus may be presumptively diagnosed. We explain our reasons for making this addition and the other changes summarized above in the public comments section of this preamble.

We are also making a minor change from the NPRM in the opening paragraph of 14.00F3. The last sentence explained that we will make every reasonable effort to obtain reports of the results of laboratory testing you have had for a manifestation of HIV infection. We are not including that sentence in final 14.00F3 because it is repetitive of other provisions in these final rules and in our other regulations. See, for example, final 14.00B and current §§ 404.1512 and 416.912. Therefore, this revision is only editorial, simplifying the proposed rule without changing any requirements.

In final 14.00F4,

HIV infection manifestations specific to women,

we incorporate the information in prior 14.00D5. In final 14.00F4a,

General,

we incorporate the first paragraph of prior 14.00D5, while in final 14.00F4b,

Additional considerations for evaluating HIV infection in women,

we incorporate the second paragraph of prior 14.00D5. Except for adding paragraph designations and headings and minor editorial changes (including changes that are reflected in the paragraph designations of the listings explained below), the final provisions are the same as in the prior rules.

In final 14.00F5,

Involuntary weight loss,

we incorporate the last paragraph of prior 14.00D2 with nonsubstantive editorial changes, including a change that reflects the redesignation of prior

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

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

In final 14.00G, we explain how we consider the effects of treatment for all three categories of immune system disorders; that is, autoimmune disorders, immune deficiency disorders, and HIV infection. The new section addresses in one place issues of treatment that are common to all three types of immune system disorders as well as issues of treatment that are unique to each type of disorder, including treatment that is specifically for HIV infection. We did not remove any guidance about treatment for HIV infection that is still relevant, but instead we moved it to this new section. In fact, we expanded and updated our rules to reflect what has been learned in applying different treatments for HIV infection since we published the prior rules. The provisions for addressing both the positive effects and negative side effects of treatment in individuals who have autoimmune disorders and immune deficiency disorders, other than HIV infection, are new in these final listings and, we believe, provide useful adjudicative guidance that was lacking in the prior rules.

Final section 14.00G has six subsections. The first two (final 14.00G1 and 14.00G2) and the last one (final 14.00G6) are applicable to all immune system disorders. Final 14.00G3-14.00G5 provide guidance specific to each of the three main types of immune system disorders: Autoimmune disorders (final 14.00G3), immune deficiency disorders, excluding HIV infection (final 14.00G4), and HIV infection (final 14.00G5).

In final 14.00G1,

General,

we incorporate the first and fifth sentences of prior 14.00D7. We believe that this guidance has general applicability to all immune system disorders, not just HIV infection. We first explain that we consider the effectiveness of your treatment on your signs, symptoms, and laboratory findings, and the negative side effects of your treatment on your functioning. We also explain that we will make every reasonable effort to obtain a specific description of the treatment you receive. Then, we list eight factors we consider when we evaluate your treatment. They are mostly based on factors we mentioned in the prior rule, but we expanded the list, and in some cases clarified the factors that were in the prior rules. For example, instead of referring only to the “dosage [and] frequency of administration” of your treatment, we refer to “the intrusiveness and complexity of your treatment (for example, dosing schedule, need for injections).” In final 14.00G1e, we also introduce the term “variability of your response to treatment,” a concept we addressed for HIV infection in prior 14.00D7 but that we believe is of particular importance in considering the effects of treatment in all individuals with immune system disorders. We explain this concept in more detail in final 14.00G2.

Final 14.00G1f is new. It describes the interactive and cumulative effects of treatments for immune system disorders and other disorders that persons with immune system disorders may also have. We explain that the effects of these treatments taken together may be greater than they would be if we considered them separately, and we provide an example of treatment for HIV infection together with treatment for hepatitis C. Final 14.00G1g is also new. It explains that we will also consider the duration of your treatment. Final 14.00G1h is a catchall for other relevant factors we have not listed in 14.00G1a-14.00G1g.

In final 14.00G2,

Variability of your response to treatment,

we explain what we mean by this factor in terms of both HIV infection and other immune system disorders. The final rule is based on the language of the second paragraph in prior 14.00D7 and the second sentence of the third paragraph of that section. However, we are expanding that guidance and applying it to all other immune system disorders in addition to HIV infection. For example, we explain in a general way applicable to all immune system disorders that some individuals may show an initial positive response to drug treatment (or a combination of drugs), but the initial positive response may be followed by a decrease in the effectiveness of the medication.

We provide more specific information about treatment of autoimmune disorders in final 14.00G3,

How we evaluate the effects of treatment for autoimmune disorders on your ability to function.

This final rule repeats the rule in the fifth paragraph of prior 14.00B that we consider the adverse effects that may result in loss of function when we evaluate the effects of your treatment for your autoimmune disorder(s). We expanded this guidance to include more examples of potential chronic adverse effects of steroid treatment and to explain that the side effects of some medications may be acute or long-term. We add a provision that recognizes that the medications used in the treatment of autoimmune disorders may have effects on mental function, including cognition (memory), concentration, and mood.

Final 14.00G4,

How we evaluate the effects of treatment for immune deficiency disorders, excluding HIV infection, on your ability to function,

is new. As in final 14.00G3, we repeat the principle that we will consider the side effects of your treatment when we evaluate your ability to function. We cite intravenous immunoglobulin and gamma interferon therapy as examples of treatment you may be receiving. We also provide examples of side effects of treatment for immune deficiency disorders, including physical symptoms (such as severe fatigue and headaches), clinical signs (such as high blood pressure and joint swelling), or limitations in mental function, including cognition, concentration, and mood.

Final 14.00G5,

How we evaluate the effects of treatment for HIV infection on your ability to function,

is in two parts. In final 14.00G5a,

General

, as in final 14.00G3 and 14.00G4, we repeat the principle from prior 14.00D7 that we consider the side effects of antiretroviral treatment and treatment for the manifestation of HIV infection on your ability to function. We expand the guidance to provide examples of the physical and mental side effects of antiretroviral drugs. We also note that the symptoms of HIV infection and the side effects of medications may be indistinguishable, and that we will consider your functional limitations whether they are a result of your symptoms or signs of HIV infection or the side effects of your treatment.

We made two changes in final 14.00G5a in response to a public comment on the NPRM. We added a parenthetical reference to “fat

redistribution, such as ‘buffalo hump’.” “Fat redistribution” is another name for lipodystrophy, which we had included in the proposed rule, and “buffalo hump” is a kind of lipodystrophy. We also expanded the last sentence of the paragraph to explain that we consider functional limitations from signs of HIV infection as well as from symptoms. We explain our reasons for these changes in the public comments section of this preamble.

In final 14.00G5b,

Structured treatment interruptions,

we provide new guidance specifically about structured treatment interruptions (STIs, also called drug holidays) in individuals with HIV infection. The guidance explains that STIs are part of a prescribed treatment plan; therefore, they do not show that an individual is failing to follow treatment or in themselves establish that an individual's impairment is not as severe as alleged.

In final 14.00G6,

When there is no record of ongoing treatment,

we explain how we evaluate the medical severity and duration of your immune system disorder when you have not received ongoing treatment or have not had an ongoing relationship with any treatment source despite the existence of a severe impairment(s). The provision is based on a standard provision we include in most other body system listings; for example, 1.00H3 in the musculoskeletal system, the third paragraph of 3.00A in the respiratory system, and the third paragraph of 4.00B3 in the cardiovascular system. We also explain that if you have just begun treatment and we cannot decide whether you are disabled based on the evidence we have, we may need to wait to determine the effect of your treatment. We explain that there is no set period because how long we may need to wait will depend on the facts of your individual case. This is consistent with the guidance we provided in the last two sentences of the third paragraph in prior 14.00D7, which explained that decisions about the impact of treatment should be based on a sufficient period of treatment to permit proper consideration of the temporary or long-term effects of the treatment.

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

Final 14.00H is new. In it, we explain that we will evaluate the impact your symptoms have on your ability to function when the evidence of your immune system disorder(s) shows that you have a medically determinable impairment that could reasonably be expected to produce your symptoms.

We added a sentence in the final rule in response to a public comment we describe later in this preamble. The sentence explains that we will not draw any inferences about your symptoms and their functional effects from the fact that you do not receive treatment or you are not following treatment without considering all of the relevant evidence in your case record, including any explanations you provide that may explain why you are not receiving or following treatment. As we explain in more detail later, the sentence is based on a provision in Social Security Ruling (SSR) 96-7p. We also clarified the heading in the final rule by listing the two constitutional symptoms, severe fatigue and malaise, instead of referring to “constitutional symptoms.”

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

We indicated in the ANPRM that we would not summarize or respond to the public comments (68 FR 24897). However, there was one theme that was common to many of the letters and e-mails and that was raised repeatedly at our two outreach meetings by the medical specialists, advocates for persons who have immune system disorders, and individuals with immune system disorders: The functional impact of immune system disorders, and the inadequacy of the immune system rules to address that impact, especially for immune system disorders other than HIV infection. This issue was raised so often, and as a matter of such great public interest, that we believe that it will be helpful to summarize briefly what commenters said to help explain why we are adding new rules for evaluating functioning in these listings.

Many commenters said that we should recognize how immune system disorders can affect an individual's functioning. Many individuals described physical symptoms, such as pain, fatigue, and malaise, as well as mental symptoms, including loss of memory, loss of concentration, and depression. Commenters stressed that these symptoms could be very severe. A number of persons indicated that the fatigue associated with these disorders was not merely a feeling of tiredness but a more profound and debilitating experience. Many individuals also noted that the impairments could be both episodic and variable in intensity, with some individuals experiencing “good” or relatively good days interspersed with days in which they were unable to function. They pointed out that there was a need for the rules to recognize the longitudinal effect of these episodic limitations on the ability to work. Other persons pointed out that there is often comorbidity of immune system disorders, that is, many persons have features of more than one immune system disorder. In those cases, the combination of symptoms and limitations have a multiplication effect in the individual's overall condition that is worse than simply adding the individual effects of the symptoms and limitations to each other. These commenters said that under the prior listings there is no adequate way to assess these multiplied effects. Many commenters also pointed out the effect that stress can have on the medical condition and symptomatology of individuals who have immune system disorders. Other individuals described the debilitating effects of treatment, not only the side effects, but sometimes the need to follow a very rigorous and time-consuming schedule of treatment that in itself can be limiting.

A number of the commenters pointed with approval to the provisions of prior listing 14.08N and the text in prior 14.00D8 that explains that listing. These individuals thought that the provisions should not be confined to persons who have HIV infection but should be extended to individuals with other kinds of immune system disorders who may be continuously limited by their symptoms and other manifestations, frequently become ill, have periodic manifestations, or have the kinds of serious limitations described in those rules. They urged us to consider extending such criteria to all listed immune system disorders to ensure that we do not overlook individuals who do not necessarily have the objective evidence needed to meet the other criteria in the listings but who may still be disabled.

As we have noted, in these final rules we are significantly expanding our guidance about specific immune system disorders and the effects of treatment. We also agree with those commenters on the ANPRM and at the public outreach meetings who suggested that we include the same kind of criteria for evaluating the overall functional impact of other immune system disorders as we provided in prior listing 14.08N for persons who have HIV infection. Therefore, we are adding criteria similar to those in prior listing 14.08N (final listing 14.08K) for each of the listed impairments in this body system. The final listings for evaluating functioning for other immune system disorders are 14.02B, 14.03B, 14.04D, 14.05E, 14.06B, 14.07C, 14.09D, and 14.10B. We are also redesignating prior listing 14.08N as

final 14.08K for reasons we explain below.

Final 14.00I is the section of the introductory text that explains the listings that include functional criteria. It corresponds to prior 14.00D8, but we revised it so that it applies to all of the new final listings that include functional criteria, not just the listing for HIV infection (prior listing 14.08N).

Like prior 14.00D8, final 14.00I includes eight paragraphs. Except as described below, we revised each paragraph so that it applies not only to HIV infection but to the other immune system disorders as well. For example, in the first paragraph of prior 14.00D8 we explained that prior listing 14.08N (final listing 14.08K) established standards for evaluating manifestations of HIV infection that do not meet the criteria of any of the preceding listings within 14.08; that is, prior listings 14.08A-14.08M. We also explained that we used prior listing 14.08N both for manifestations that were listed in the preceding listings within 14.08 and for manifestations that were not listed at all. We have modified this language so that it applies to all of the immune system disorders within this body system. We also made minor editorial changes throughout the paragraphs.

The following are other changes we are making in this section.

In final 14.00I2, we are removing the first sentence in the second paragraph of prior 14.00D8. That sentence explained that, for individuals with HIV infection, we assessed listing-level severity under prior listing 14.08N based on the functional limitations imposed by the impairment. We believe that this point is already made in final 14.00I1 and that it is unnecessary to repeat it in final 14.00I2. We are revising the second sentence, which said that we must consider the full impact of “signs, symptoms, and laboratory findings” on the individual's ability to function. We believe that this guidance may not have clearly explained what we intended. Therefore, we are revising it to explain that when we use one of the listings cited in final 14.00I1, we will consider all relevant information in your case record to determine the full impact of your immune system disorder(s) on your ability to function on a sustained basis.

In final 14.00I3-14.00I8, which correspond to the last six paragraphs in prior 14.00D, we are updating our rules to make their language more consistent with our other rules that define the term “marked” and the areas of functioning. However, these changes are not intended to be substantively different from the prior rules. We are also including references to both pain and severe fatigue throughout final 14.00I6-14.00I8 as symptoms that may cause limitations. The prior rules were not consistent in this regard.

We added guidance in final 14.00I3 in response to public comments on the NPRM. The guidance clarifies that your impairment will satisfy the criterion for “repeated” manifestations regardless of whether you have the same kind of manifestation repeatedly, all different manifestations, or a combination of some manifestations that are the same and some different; for example, two of the same kind of manifestation and one different one. You must only have the required number of manifestations with the frequency and duration required in this section. This is not a change in meaning from the proposed rules, but a clarification of our intent. In response to another comment, we also clarify that the manifestations must occur within the period covered by your claim.

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

Final 14.00J1 and 14.00J3 replace the guidance we provided in the first and third paragraphs of prior 14.00D6. As in other provisions throughout the introductory text, we are revising the language to make it apply generally to all immune system disorders, not just HIV infection. Also, we are removing guidance that is already covered in other sections in the introductory text, such as the guidance that individuals may have signs or symptoms of a mental impairment or of another physical impairment.

Final 14.00J2 is a new section in this body system. For reasons we have already explained, we are removing reference listings—that is, listings that are met or equaled by meeting or equaling the criteria of another listing—from this body system. However, immune system disorders can have effects in virtually every body system, and we believe it is important to include guidance about those effects in the introductory text so that they are not overlooked.

Therefore, we are adding section 14.00J2 to explain that immune system disorders can have effects in other body systems; we also provide a list of examples of those effects in each of the relevant body systems with references to other body system listings. These provisions are based on language in the second paragraph of prior 14.00D6, which was relevant only to the evaluation of HIV infection, and on the reference listings we are removing. We are expanding the information that was in that paragraph to provide specific examples of impairments that may be caused by autoimmune disorders.

For example, prior listings 14.02A6 and 14.04A4 were met with evidence of SLE, systemic sclerosis, or scleroderma with “Digestive involvement, as described under the criteria in 5.00ff.” Apart from the fact that these listings were unnecessary because any individual who meets the criteria of a listing in the digestive body system (5.00) would be disabled under that listing, the guidance was not very specific. Also, in the prior rules, we included these criteria only under prior listings 14.02 and 14.04. However, other immune system disorders can have effects in the digestive system. Therefore, in final 14.00J2e, we provide that any immune system disorder can have effects in the digestive system, and we include an example of hepatitis C in addition to providing a reference to 5.00.

In these final rules, we are adding a reference to weight loss as a result of HIV infection that affects the digestive system in final 14.00J2e. We explain later in this preamble that our reason for adding this reference is to respond to public comments we received on the NPRM about HIV wasting syndrome.

Final 14.00J2k provides examples of allergic disorders (including skin disorders) that individuals with immune system disorders may have. It replaces prior 14.00C.

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

14.01—Category of Impairments, Immune System Disorders

The following is a detailed explanation of the significant changes in the final listings. Some changes are common to several listings, so we describe them first.

1. We are removing all of the reference listings from this body system for reasons we have already explained.

2. We are revising prior listings 14.02B, 14.03B, 14.04B, and 14.09D (final listings 14.02A, 14.03A, 14.04A, and 14.09B) as follows:

• We are removing the criterion for “significant, documented” constitutional symptoms or signs in each of these listings because we define the constitutional symptoms and signs in final 14.00C2. Moreover, it is unnecessary to specify “documented” because we always need to document the existence of any symptom or sign in any disability claim.

• Each of these prior listings, except prior listing 14.09D, also required you to

have all four of the constitutional symptoms or signs: Severe fatigue, fever, malaise, and involuntary weight loss. We are revising this requirement to “at least two” of the constitutional symptoms or signs, instead of all four, because we believe that the requirement in the prior listings was too severe. We believe that any individual with an autoimmune disorder involving two or more organs/body systems with one organ/body system involved to at least a moderate level of severity and who has at least two of the constitutional symptoms and signs in these listings will have an impairment that precludes any gainful activity. We have also added “involuntary” as a descriptor of weight loss in final listings 14.02A, 14.03A, 14.04A, 14.05E, 14.06A, 14.07C, 14.08K, 14.09B, and 14.10A for reasons we explained earlier in this preamble.

• In final listings 14.02A, 14.03A, and 14.04A, which correspond to prior listings 14.02B, 14.03B, and 14.04B, we are removing the reference to “lesser involvement” because we are removing the prior reference listings to which these rules refer. We also believe the phrase is unnecessary—the severity of the impairment is demonstrated by the remaining criteria.

3. As we have already noted under the explanation of final 14.00I, we are adding listings based on repeated manifestations accompanied by functional limitations and modeled after prior listing 14.08N (final listing 14.08K) for each of the other immune system disorders. The final listings are:

• 14.02B for SLE,

• 14.03B for systemic vasculitis,

• 14.04D for systemic sclerosis (scleroderma),

• 14.05E for polymyositis and dermatomyositis,

• 14.06B for undifferentiated and mixed connective tissue disease,

• 14.07C for immune deficiency disorders, excluding HIV infection,

• 14.09D for inflammatory arthritis, and

• 14.10B for Sjögren's syndrome.

Each listing requires you to have:

• The specified immune system disorder for that listing,

• Repeated manifestations of the specified immune system disorder,

• At least two of the constitutional symptoms or signs, and

• A “marked” limitation in one of three domains of functioning: Activities of daily living, maintaining social functioning, or completing tasks in a timely manner due to deficiencies in concentration, persistence, or pace.

We explain what we mean by “repeated” in final 14.00I3 and by “marked” in final 14.00I4-5.

In the final rules, we made a number of changes from the proposed rules in response to public comments on the NPRM. Chiefly, we removed from several listings the requirement that there must be manifestations “without the requisite findings in” a specified paragraph earlier in the listing; for example, proposed listing 14.02B said “without the requisite findings in [14.02]A.” Our only intent was to explain that we would use the listing criterion (for example, listing 14.02B) when you have an impairment that does not meet the requirements of the previously specified listing section (for example, listing 14.02A). However, a public comment pointed out that our language could have been confusing, and we determined that it was not necessary to have it at all. We explain in detail the public comment and our reasons for making this change throughout the final listings in the public comments section of this preamble.

The following is an explanation of the other significant changes we are making. We are also making minor editorial changes in some listings and changes to cross-references to the introductory text throughout the listings to reflect the changes to the introductory text for the final rules. We do not describe all of those changes below.

Final Listing 14.04—Systemic Sclerosis (Scleroderma)

Final listing 14.04B corresponds to prior listing 14.04C. As we have already noted, we are expanding this listing to include provisions for individuals who had a form of the disorder as children and who still have listing-level functional limitations as adults. The final listing is essentially identical to final listing 114.04, which we describe in detail later in this preamble, except that it includes references to appropriate adult rules defining “inability to ambulate effectively” and “inability to perform fine and gross movements effectively.”

We are also making minor clarifications in the language of the prior listing. Prior listing 14.04C described “[g]eneralized scleroderma with digital contractures.” We are clarifying that “digital” refers to either the toes or the fingers and are listing the effects in the toes separately from the effects in the fingers in final listings 14.04B1 and 14.04B2, respectively. We also are removing the requirement for “generalized” scleroderma (that is, systemic sclerosis) because the very serious digital contractures described in the final listings would in themselves be disabling regardless of whether the scleroderma is generalized.

Final listing 14.04C corresponds to prior listing 14.04D. We are changing “Raynaud's phenomena” in prior listing 14.04D to “Raynaud's phenomenon” for the same reason already described in the explanation of final 14.00D3. We are removing the word “[s]evere” as a descriptor of Raynaud's phenomenon in this listing because it is unnecessary given the severity of the impairment demonstrated by the remaining criteria, such as ischemia with ulcerations of toes or fingers, resulting in the inability to ambulate effectively or to perform fine and gross movements effectively. As in final listing 14.04B, we also are clarifying that “digital” refers to fingers or toes.

In final listing 14.04C, we are also revising the criteria in prior listing 14.04D to provide a better description of listing-level Raynaud's phenomenon. The criteria in prior listing 14.04D required severe Raynaud's phenomenon characterized by digital ulcerations, ischemia, or gangrene. As we noted in the NPRM, we believe that this included some individuals who did not have impairments of listing-level severity.

Therefore, in final listing 14.04C1, we provide criteria for Raynaud's phenomenon characterized by gangrene involving “at least two extremities” to establish an impairment that would preclude any gainful activity. The final rule is somewhat different from the proposed rule, which referred to fingers and toes. We clarified it in response to a public comment on the NPRM that we describe in the public comments section of this preamble. As in the NPRM, we do not require that the gangrene result in the inability to ambulate effectively or to perform fine and gross movements effectively because the presence of gangrene involving at least two extremities by itself demonstrates a very serious impairment.

In final listing 14.04C2, we provide criteria for ischemia with ulcerations of the toes or fingers that results in the inability to ambulate effectively or to perform fine and gross movements effectively; Raynaud's phenomenon characterized only by ischemia with ulcerations does not, by itself, describe an impairment that would necessarily result in an extreme loss of function. Also, ulcerations are an outcome of ischemia, so we are revising the language of the prior rule so that ischemia and ulcerations are not listed as though they are separate entities.

Final Listing 14.05—Polymyositis and Dermatomyositis

Final listing 14.05A corresponds to prior listing 14.05A. We are replacing the word “severe” as a descriptor of proximal limb-girdle weakness with the more accurate “resulting in inability to ambulate effectively or inability to perform fine and gross movements effectively, as defined in 14.00C6 and 14.00C7.” We are also changing “shoulder and/or pelvic” muscle weakness to “pelvic or shoulder” muscle weakness because either pelvic muscle weakness that results in the inability to ambulate effectively or shoulder muscle weakness that results in the inability to perform fine and gross movements effectively is sufficient in itself to show disability, and the “and” is unnecessary.

Final listing 14.05B corresponds to prior listing 14.05B1. We are removing a number of the requirements from the prior rule because we have determined that impaired swallowing with aspiration due to muscle weakness establishes a listing-level impairment. We are revising the requirement for “episodes of aspiration” to only “aspiration” because of the progressive nature of muscle weakness that results from polymyositis or dermatomyositis. Once an episode of aspiration is documented, further documentation of multiple episodes is unnecessary. In addition, we are replacing “cricopharyngeal weakness” with “muscle weakness” in final 14.05B because impaired swallowing and aspiration may result from muscles other than the cricopharyngeal muscles. Finally, we are revising the phrase “impaired swallowing with dysphagia” to “impaired swallowing (dysphagia)” because “dysphagia” means impaired swallowing.

Final listing 14.05C corresponds to prior listing 14.05B2, for individuals who have polymyositis or dermatomyositis with impaired respiration due to intercostal and diaphragmatic muscle weakness.

Final listing 14.05D, Diffuse calcinosis, is a new listing for adults that has the same criteria as final listing 114.05D for children, which we describe in detail later in this preamble. We are adding this listing for individuals who had a form of the disorder as children and who still have listing-level functional limitations as adults.

Final Listing 14.06—Undifferentiated and Mixed Connective Tissue Disease

We are changing the heading of prior 14.06 to update it and to more accurately describe the disorders we evaluate under this listing.

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

Final Listing 14.07—Immune Deficiency Disorders, Excluding HIV Infection

We are changing the heading of listing 14.07 to update its terminology and to more accurately describe the disorders we evaluate under this listing.

The prior listing was met with documented, recurrent severe infections occurring three or more times within a 5-month period. We are replacing this criterion with a more accurate and up-to-date listing. The listing is in three parts.

Final listing 14.07A is essentially the same as final listing 14.08J (prior listing 14.08M), which describes individuals with HIV infection whose immune systems are so compromised that they frequently become ill. We believe that these criteria for individuals with HIV infection are equally as applicable to individuals with other kinds of immune deficiency disorders, and that they are more inclusive than the criteria in prior listing 14.07.

As in final listing 14.08J, final listing 14.07A provides that the infections must occur three times in a 12-month period, not three times in only a 5-month period. It also more precisely explains how severe the infections need to be by requiring either resistance to treatment or a need for hospitalization or intravenous treatment. It also specifies six types of infections.

Final listing 14.07B is new. We are adding this listing to recognize that some immune system disorders are treated by stem cell transplantation. In final listing 14.07B, we state that we will consider you to be under a disability until at least 12 months from the date of transplantation and, thereafter, evaluate any residual impairment(s) under the criteria for the affected body system.

Final listing 14.07C incorporates the same functional criteria for the evaluation of repeated manifestations of immune deficiency disorders (excluding HIV infection) as in the other final listings in this body system and for the same reasons as described above.

Final Listing 14.08—Human Immunodeficiency Virus (HIV) Infection

Except as described below, we are not making any changes to the criteria in listing 14.08. As noted in the NPRM, we carefully considered the advances in treatment and consequent increases in longevity that have occurred since we published the prior rules in 1993. Based on this review, we did not believe that there had been sufficient progress in the treatment and control of HIV infection to warrant any change in these rules at that time. However, as a result of public comments we received on the NPRM, we now believe that some changes may be appropriate. Therefore, while final listing 14.08 is substantively the same as proposed listing 14.08, we are publishing separately an ANPRM in today's edition of the

Federal Register

inviting comments and suggestions on how to update and revise our listing for HIV infection. We will consider the comments and suggestions that we receive in response to the ANPRM, as well as our adjudicative experience and additional information about advances in medical knowledge, treatment, and methods of evaluating HIV infection. If we determine that listing 14.08 should be revised, we will publish for public comment an NPRM that will propose specific revisions to the listing.

As already noted, we are removing reference listings throughout this body system, including the reference listings in listing 14.08. This results in the removal of several specific listings within 14.08 and the redesignation of some of the prior listings; for example, prior listing 14.08N has become final listing 14.08K. Where we are removing a reference listing, however, we have ensured that we provide guidance in the introductory text about where to evaluate the impairment. For example, prior listing 14.08A4, for HIV infection with syphilis or neurosyphilis, was a reference listing that said only to consider the impairment under the criteria for the affected body system, such as 2.00 (special senses and speech), 4.00 (cardiovascular system), or 11.00 (neurological). Although we are removing this reference listing, we include this same guidance in final 14.00J2l.

We are also clarifying some of the rules. In final listing 14.08B2, we are reorganizing the language from prior listing 14.08B2 to make it clearer that we evaluate under this listing candidiasis involving the esophagus,

trachea, bronchi, or lungs, or at another site other than the skin, urinary tract, intestinal tract, or oral or vulvovaginal mucous membranes. We are moving prior listing 14.08C2, for PCP, from the listing for protozoan and helminthic infections to the listing for fungal infections because the organism that causes PCP is now known to be a fungus. We redesignate it as final listing 14.08B7.

We are redesignating prior listing 14.08N as final listing 14.08K. We are expanding our guidance on manifestations we evaluate under final listing 14.08K by adding “pancreatitis, hepatitis, peripheral neuropathy, glucose intolerance, muscle weakness, cognitive or other mental limitation” as new examples. We are also expanding our list of signs and symptoms by adding “nausea, vomiting, headaches, or insomnia.”

We made minor changes to the language of the functional criteria in final listing 14.08K from the language in prior listing 14.08N. For example, we replaced the words “restriction” in prior listing 14.08N1 and “difficulties” in prior listings 14.08N2 and 14.08N3 with the word “limitation” in final listings 14.08K1, 14.08K2, and 14.08K3. We made this change because “limitation” is a more accurate description for the functional criteria in these listings.

We are making a number of changes from the proposed rule in response to public comments on the NPRM and for editorial reasons. The changes are in:

• Final listing 14.08B2, in which we made a minor editorial correction to remove a redundant word;

• Final listing 14.08B7, in which we removed the word “

carinii

” and the parenthetical “

jiroveci

” from the name of “

Pneumocystis

pneumonia” in response to a public comment on the NPRM;

• Final listing 14.08E4, in which we revised the criterion from “squamous cell carcinoma of the anus” to “squamous cell carcinoma of the anal canal or anal margin” in response to a public comment on the NPRM;

1

1

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

• Final listing 14.08H, in which we clarified that the 10 percent loss of weight from baseline may be calculated in pounds, kilograms, or by body mass index (BMI) in response to a public comment on the NPRM;

• Final listing 14.08J, in which we removed an unnecessary comma; and

• Final listing 14.08K, in which we changed the reference to “fatigue” to “severe fatigue” and a reference to a “mental impairment” to a “mental limitation” in response to public comments on the NPRM, and removed the proposed cross-reference to 14.00I5. The removal of the cross-reference is only editorial. The reference was unnecessary, incomplete (the term “marked” for the various domains is also defined in final 14.00I6, 14.00I7, and 14.00I8), and inconsistent with other sections of the proposed immune disorder listings which contained the same severity criteria but did not include this cross-reference.

We provide detailed explanations of the changes we made in response to public comments on the NPRM and our reasons for making them in the public comments section of this preamble.

Final Listing 14.09—Inflammatory Arthritis

We are redesignating prior listing 14.09D as final listing 14.09B, prior listing 14.09B as final listing 14.09C1, and prior listing 14.09E as final listing 14.09C2 to put these listings in a more logical order. In the final rules, listing 14.09A describes persistent inflammation or deformity of major peripheral joints that alone is disabling, while listing 14.09B describes disability with lesser inflammation or deformity of major peripheral joints together with organ involvement and constitutional symptoms or signs. Final listing 14.09C describes listing-level inflammatory arthritis of the spine. Final listing 14.09C1 describes disability based only on fixation (ankylosis) of the spine, while final listing 14.09C2 describes disability based on a lesser degree of ankylosis of the spine with organ involvement. Final listing 14.09D is the same functional listing we include in all of the final immune system disorders listings and applies to inflammatory arthritis affecting any joints.

Final listing 14.09A corresponds to prior listing 14.09A. We are removing the requirement for a history of joint pain, swelling, and tenderness from this listing because it is unnecessary. (We do refer to joint pain, swelling, and tenderness in final 14.00D6a as possible signs and symptoms of the disorder.) Persistent joint inflammation or deformity in one or more major peripheral weight-bearing joints resulting in the inability to ambulate effectively, or persistent joint inflammation or deformity of major peripheral joints in both upper extremities resulting in inability to perform fine and gross movements effectively, is in itself indicative of an impairment that would preclude any gainful activity. For the same reasons, we are also removing the requirement for “signs on current physical examination.” We do not need signs of joint inflammation on a current physical examination when we have medical evidence documenting that you have inflammatory arthritis that results in the inability to ambulate effectively or inability to perform fine and gross movements effectively. Also, because of the episodic nature of inflammatory arthritis, a current physical examination could show a brief period of improvement for a few days even though your longitudinal medical records may show persistent joint inflammation that results in the inability to ambulate effectively or inability to perform fine and gross movements effectively.

As we noted under the explanation of final 14.00D6e, we are revising listing 14.09A in response to a public comment on the NPRM so that there is no longer a need to use listing 1.02 or 1.03 in cases involving inflammatory arthritis. Final listing 14.09 (and final listing 114.09) will apply to all individuals who have listing-level limitations as a result of inflammatory arthritis. The revised listing includes essentially the same requirements as listings 1.02 and 1.03 of the musculoskeletal listings.

Because of this, we are changing the structure of final listing 14.09A to provide separate criteria for inflammatory arthritis that involves one or more major peripheral weight-bearing joints (final listing 14.09A1) and inflammatory arthritis involving one or more major peripheral joints in both upper extremities (final listing 14.09A2), with appropriate severity criteria for each. We define the “major peripheral joints” in final 14.00C8.

Final listing 14.09B corresponds to prior listing 14.09D. The revisions in final 14.09B are similar to those in final listing 14.09A for the same reasons and to make it clearer that this listing requires joint inflammation in one or more major peripheral joints. Final 14.09B continues to require less joint involvement than in A, but we no longer require “lesser extra-articular features than in C” because “C” refers to prior reference listing 14.09C, which we have removed. Final listing 14.09B1 corresponds to prior listing 14.09D2 with nonsubstantive editorial changes to make it consistent with how we present this criterion throughout these listings. Final listing 14.09B2 corresponds to prior listing 14.09D1 except that we have removed the phrase “significant, documented” for reasons we have already explained. We are also correcting an error in prior listing 14.09D1. The explanatory abbreviation, “e.g.” (for example) in prior listing

14.09D1 inaccurately indicated that the four constitutional symptoms or signs, that is, severe fatigue, fever, malaise, and involuntary weight loss, were only examples when they are in fact a complete list. Consistent with changes in other final listings, we are requiring at least two of the constitutional symptoms or signs because we believe that the criteria in final listing 14.09B are indicative of an impairment that precludes any gainful activity.

Final listing 14.09C1 corresponds to prior listing 14.09B. We are reorganizing the criteria and removing the requirements for “diagnosis established by findings of unilateral or bilateral sacroiliitis (e.g., erosions or fusions)” and “[h]istory of back pain, tenderness, and stiffness” because these findings are unnecessary. We believe ankylosing spondylitis or other spondyloarthropathies with ankylosis of the dorsolumbar or cervical spines at 45° or more of flexion documented as required in final listing 14.09C1 are in themselves indicative of an impairment that precludes any gainful activity.

Final listing 14.09C2 corresponds to prior listing 14.09E. We are reorganizing this listing to make it more consistent with the structure and criteria that we use in the final listings for other autoimmune disorders. We are removing the phrase “with lesser deformity than in B,” which describes a deformity that is less than the fixation “of the dorsolumbar or cervical spine at 45° or more of flexion” under prior listing 14.09B, and replacing it with fixation “at 30° or more of flexion (but less than 45°).” We believe that this is a clearer and more specific criterion that helps to provide greater uniformity in adjudications under this listing. We are removing the phrase “lesser extra-articular features than in C” because it refers to prior reference listing 14.09C, which we are removing. We also are removing the phrase “with signs of unilateral or bilateral sacroiliitis” because the criteria in the final listing would be sufficient to show listing-level severity without this requirement, and the phrase “with the extra-articular features described in 14.09D” because it is unnecessary.

Final Listing 14.10—Sjögren's Syndrome

Final listing 14.10 is new. We are adding it in response to comments we received before we developed the NPRM indicating that Sjögren's syndrome is distinct from other immune system disorders and that it has unique aspects that the prior immune system listings did not address.

Although individuals with Sjögren's syndrome were able to qualify under prior listings 14.03 and 14.09 and other listings, we believe that it is now appropriate to list Sjögren's syndrome separately in these listings. We are using the same two listing criteria for establishing listing-level severity as in the other final listings for autoimmune disorders because Sjögren's syndrome is an autoimmune disorder that can cause the same kinds of constitutional symptoms and signs as other autoimmune disorders, and because it can be as functionally limiting as other autoimmune disorders. Final listing 14.10A is the same as final listings 14.02A, 14.03A, 14.04A, and 14.06A, and final listing 14.10B is the same as final listings 14.02B, 14.03B, 14.04D, 14.05E, 14.06B, and 14.09D. As already noted, we also provide a new separate section in the introductory text that describes the unique features of Sjögren's syndrome, final 14.00D7.

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

As in final 14.00 in the adult rules, we are changing the name of this body system to “Immune System Disorders.”

Except for minor editorial changes, we have repeated much of the introductory text of final 14.00 in the introductory text of final 114.00. This is because the same basic rules for establishing and evaluating the existence and severity of immune system disorders in adults also apply to children. Because we have already described these provisions under the explanation of final 14.00, the following discussions describe only those provisions that are unique to the childhood rules or that require further explanation. We describe only the major provisions. For example, we do not summarize minor editorial changes that refer to “children” instead of adults or to the policy of “functional equivalence” instead of RFC assessment and steps in the adult sequential evaluation process.

Also, where appropriate in the introductory text of final 114.00, we have made an editorial change from the prior rules in the terms we use to identify the age categories of children in the introductory text of prior 114.00 to be consistent with the terms we use in the introductory text of current 112.00, Mental disorders. For example, in final 114.00F1b(ii), we use “newborn and younger infants (birth to attainment of age 1)” instead of “an infant 12 months of age or less” as in prior 114.00D3b(i).

Finally, we have changed the part B final rules from the NPRM in the same way that we changed the part A final rules from the NPRM whenever those proposed rules were the same.

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

In final 114.00A1b, we incorporate the first sentence in the last paragraph of prior 114.00B, which explains that immune system disorders may affect growth, development, attainment of age-appropriate skills, and performance of age-appropriate activities in children. We are revising the sentence by adding the phrase “or their treatment.” We are also removing the phrase “attainment of age-appropriate skills” because it is redundant of “development.”

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

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

Final 114.00D parallels the structure and content of final 14.00D in the adult rules, except where the features commonly associated with the autoimmune disorders in these listings differ in children from adults.

In final 114.00D2,

Systemic vasculitis (114.03)

, as in prior 114.00C3, we provide guidance (in final 114.00D2a(ii)) on how we evaluate Kawasaki disease and add guidance about anaphylactoid purpura (Henoch-Schoenlein purpura). Also, in final 114.00D2a(ii), we do not use the example of giant cell arteritis (temporal arteritis) that is in final 14.00D2a(ii) because this disorder occurs almost exclusively in individuals over 50 years of age.

In final 114.00D3c,

Localized scleroderma (linear scleroderma or morphea)

, we describe features of focal forms of scleroderma in children. These disorders occur primarily in children and are more common than systemic sclerosis in children. In final

114.00D3c(i), we explain that the extent of involvement and the location of the lesions are important factors in determining the limitations resulting from scleroderma. We also note that it may be appropriate to evaluate the limitations resulting from these impairments under the musculoskeletal listings (101.00).

In final 114.00D3c(ii), we describe features of isolated morphea of the face and explain that it may be more appropriate to evaluate the limitations from these disorders under the affected body system, such as special senses and speech (102.00) or mental disorders (112.00). We have made a minor correction in the final rule. In the NPRM, we indicated that it would be more appropriate to evaluate the limitations from these disorders only under the special senses or mental disorders listings. However, we explained in the preamble that these body systems were only examples of body systems that might be affected. In the final rule, we are clarifying that the body systems we cite are only examples. We have made the same correction in part A.

In final 114.00D3c(iii), we describe musculoskeletal and respiratory features of chronic variants of these syndromes and explain that it is appropriate to evaluate the limitations from these disorders under the musculoskeletal listings (101.00) or respiratory system listings (103.00).

In final 114.00D4,

Polymyositis and dermatomyositis (114.05)

, we note (in final 114.00D4a,

General

) that polymyositis occurs rarely in children and describe the features of dermatomyositis that occur differently in children than in adults.

In children, polymyositis and dermatomyositis usually do not occur in association with malignancies. For this reason, we do not include a reference to malignancy or provide guidance that we will evaluate malignancies under the malignant neoplastic diseases listings (113.00) in final 114.00D4, as we do for adults in final 14.00D4. However, unlike in the adult rules, we include a reference to calcinosis for children in this section. Calcinosis is primarily an outcome of juvenile dermatomyositis; when adults with dermatomyositis have calcinosis, it is generally because they have had the condition since childhood. For this reason, we refer to calcinosis only in the introductory text for children, final 114.00D4. However, we include a criterion for diffuse calcinosis in final listing 14.05D (as well as final listing 114.05D) for adults who have the condition. Also, when dermatomyositis involves other organs or body systems, we evaluate the involvement under the affected body system.

In final 114.00D4b,

Documentation of polymyositis or dermatomyositis

, we note that magnetic resonance imaging (MRI) showing muscle inflammation or vasculitis provides additional evidence of childhood dermatomyositis. We did not provide this guidance in final 14.00D4b because MRI findings are not considered diagnostic of dermatomyositis in adults. Similar to final 14.00D4b, we added two sentences to the final rule to indicate that when the results of electromyography, muscle biopsy, or MRI are in your medical records we will make every reasonable effort to obtain them, but that we will not purchase any of these tests.

In final 114.00D4c(i), we explain how to evaluate polymyositis and dermatomyositis under the listings in newborn and younger infants.

In final 114.00D5,

Undifferentiated and mixed connective tissue disease (114.06)

, we note (in final 114.00D5a,

General

) that the most common pattern of undifferentiated autoimmune disorders in children is mixed connective tissue disease (MCTD). In final 114.00D5b,

Documentation of undifferentiated and mixed connective disease

, we note diagnostic laboratory findings specifically for children with MCTD and that the clinical findings are often suggestive of SLE or childhood dermatomyositis. We also note that many children later develop features of scleroderma.

In final 114.00D6,

Inflammatory arthritis (114.09)

, we incorporate (in final 114.00D6a,

General

) guidance from prior 114.00C2 and 114.00E. We explain that we evaluate growth impairment resulting from inflammatory arthritis under the criteria in 100.00. In final 114.00D6b,

Inflammatory arthritis involving the axial spine

(

spondyloarthropathy

), we incorporate the second sentence in prior 114.00E and revise some of the examples of disorders that may be associated with inflammatory spondyloarthropathies involving the axial spine with disorders that are more common in children.

Prior 114.00E6 provided that the fact that a child is dependent on steroids, or any other drug, for the control of inflammatory arthritis is, in and of itself, insufficient to find disability. It explained that advances in the treatment of inflammatory connective tissue disease and in the administration of steroids for its treatment have corrected some of the previously disabling consequences of continuous steroid use. Although this statement is still true, we are not including this provision of prior 114.00E6 in these final rules because we believe we no longer need it in the introductory text for the listings.

We added prior 114.00E6 in 2002 (66 FR at 58022 and 58045). It was important when we added it because the listings prior to the revisions we made in 2002 included a listing (prior listing 101.02B) that said that all children with rheumatoid arthritis who were dependent on steroids were disabled. We removed that listing in 2002, explaining that, although the prior listing was appropriate when we first published it, advances in treatment and other reasons had made it obsolete (66 FR at 58022). Thus, the paragraph in the introductory text served as a reminder that we no longer had that listing and that it was no longer appropriate to presume disability based on steroid use alone. Now that several years have passed since we removed the prior listing, we do not believe that we need this reminder any longer. However, in final 114.00G3, we continue to state that we will consider the adverse side effects of treatment, including the effects of corticosteroids, to ensure that our adjudicators remember to consider the side effects of steroids and any other treatment an individual might have.

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

Final 114.00F parallels the structure and content of final 14.00F in the adult rules, except where the features commonly associated with HIV infection differ in children from adults.

Final 114.00F1a,

Definitive documentation of HIV infection

, corresponds to 114.00D3a in the prior rules and 14.00F1a in the final rules. In final 114.00F1a(i), we are lowering the age for using HIV antibody tests from the 24 months of age or older that was in prior 114.00D3a(i) to 18 months or older. Current clinical practice now accepts these tests beginning at 18 months of age.

In final 114.00F1a(iv), we clarify the provision in prior 114.00D3a(ii) by explaining that a specimen that contains HIV antigen may be used to establish the diagnosis of HIV infection in a child age 1 month or older.

Final 114.00F1b,

Definitive documentation of HIV infection in children from birth to the attainment of 18 months

, corresponds to the second paragraph in prior 114.00D3b,

Other acceptable documentation of HIV infection in children

. We are moving this information and revising the age cutoff to 18 months to recognize that laboratory values we previously considered to be “other acceptable

documentation” of HIV infection are now considered definitively diagnostic in children from birth to age 18 months who have tested positive for HIV antibodies.

In final 114.00F1b(i), we add “One or more of the tests listed in F1a(ii)-F1a(vii)” of final 114.00F1a because these tests are accepted as diagnostic of HIV infection.

In final 114.00F1b(iii), we change “12 to 24 months of age” in current 114.00D3b(ii) to “12 to 18 months of age” based on how these findings are used in current clinical practice.

In final 114.00F1b(v), we specify that a severely diminished immunoglobulin G (IgG) level is “< 4g/l or 400 mg/dl.” However, we do not provide an IgG level for greater than normal range for age due to the variability in the higher normal range of IgG level in children by age. There is consistency in the normal lower average range in children, so we are able to specify levels for severely diminished IgG.

Final 114.00F1c,

Other acceptable documentation of HIV infection

, corresponds to prior 114.00D3b and final 14.00F1b. We are removing the first paragraph in prior 114.00D3b, which explained that HIV infection is not documented in children under 24 months of age by a serum specimen containing HIV antibodies. All infants who have HIV antibodies are now tested to determine definitively whether they have HIV infection.

In final 114.00F2,

CD4 tests

, we add more detailed guidance to the second paragraph of prior 114.00D4a by specifying that the extent of immune depression correlates with the level of CD4 counts (relative to the age of the child), and that by age 6, CD4 levels become comparable to adult CD4 levels.

In final 114.00F3b,

Other acceptable documentation of the manifestations of HIV infection

, we explain, in 114.00F3b(i) for PCP and in 114.00F3b(ii) for CMV disease, that a CD4 count below 200 in children 6 years of age or older is supportive evidence of a presumptive diagnosis of these manifestations.

Final 114.00F4,

HIV manifestations specific to children

, corresponds to prior 114.00D5,

HIV in children

. In final 114.00F4a,

General

, we are removing the second sentence in prior 114.00D5. That sentence explained that survival times were shorter for children who were infected in the first year of life than they were for older children and adults. However, due to advances in medical treatment this is no longer the case. The second sentence of final 114.00F4a is based on the first paragraph in prior 114.00D5.

In final 114.00F4b,

Neurologic abnormalities

, we make some nonsubstantive editorial changes to the second paragraph in prior 114.00D5 in which we explained that the methods of identifying and evaluating neurological abnormalities vary depending on a child's age. We also replace “acquisition” with “onset” in the last sentence of final 114.00F4b because a sudden “onset” of a new learning disability is medically a more accurate description of how this neurologic abnormality would manifest in a child with HIV infection.

In final 114.00F4c,

Bacterial infections

, we incorporate the last two paragraphs in prior 114.00D5. We make only nonsubstantive editorial changes, including removing text that only repeats criteria from the listings.

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

In final 114.00G2,

Variability of your response to treatment

, we use an example of a child who develops otitis media instead of pneumonia or tuberculosis as we do in final 14.00G2 for an adult because otitis media is more common in children.

In final 114.00G3,

How we evaluate the effects of treatment for autoimmune disorders on your ability to function

, we use examples of impaired growth and osteopenia instead of osteoporosis as we do in final 14.00G3 because impaired growth and osteopenia are more common in children.

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

As in the adult rules, we are adding listings based on functional criteria to each of the listings in the immune system in addition to those that are already in listing 114.08. Final 114.00I—

How do we use the functional criteria in these listings?

—corresponds to prior 114.00D8 and provides guidance for applying the listings based on functional criteria in all of the final childhood listings. We revised the prior language to reflect the fact that there are now functional listings for each of the listed impairments in this body system and for consistency with adult rules where appropriate.

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

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

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

Final 114.01—Category of Impairments, Immune System Disorders

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

The following is a description of the significant changes in part B when they are different from the changes we made in part A or require additional explanation.

Final Listing 114.04—Systemic Sclerosis (Scleroderma)

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

Final listings 114.04B3 and 114.04B4 correspond to prior listing 114.04B2, the listing for “[m]arked destruction or marked atrophy of an extremity.” We are revising the prior rules to:

• Remove the word “marked,”

• Change the criterion for “destruction” to “irreversible damage,”

• Require both atrophy and irreversible damage in one or both lower extremities or both upper extremities, and

• Require either inability to ambulate effectively or to perform fine and gross movements effectively.

We are removing the word “marked” because we use it in various other listings and other regulations to describe a particular measure of functional limitations, and it does not describe what we intend in this listing. We are replacing the criterion for “marked destruction” with a criterion for “irreversible damage” because it is a more accurate medical description of this complication of systemic sclerosis. We are requiring both atrophy and irreversible damage because we would not expect either of these findings alone to establish an impairment that results in marked and severe functional limitations in every case. Finally, we are requiring “inability to ambulate effectively” or “inability to perform fine or gross movements effectively” to establish an impairment that is of listing-level severity, consistent with other listings.

Final listing 114.04C, Raynaud's phenomenon, is a new childhood listing and has the same criteria as in final listing 14.04C for adults.

Final Listing 114.05—Polymyositis and Dermatomyositis

We are removing prior listing 114.05B1 because multiple joint contractures are not typically a part of the disease process of polymyositis or dermatomyositis in children. However, if this should occur, we would evaluate whether your polymyositis or dermatomyositis with multiple joint contractures meets or medically equals the criteria in final listing 114.05E, medically equals the criteria in another listing, such as final listing 114.05A, or functionally equals the listings.

In final listing 114.05D, we are revising prior listing 114.05B2 by replacing “cutaneous calcification” with “calcinosis.” We are making this change because “calcification” describes the normal process by which calcium salts are deposited in bone, and “calcinosis” describes the abnormal deposits of calcium salt in body tissues as we intend by this criterion. We are also replacing “formation of an exoskeleton” with “limitation of joint mobility or intestinal motility” because it is a better description of the known complications of dermatomyositis in children.

Final Listing 114.07—Immune Deficiency Disorders, Excluding HIV Infection

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

Final Listing 114.08—Human Immunodeficiency Virus (HIV) Infection

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

Final Listing 114.10— Sjögren's Syndrome

We are adding listing 114.10 to evaluate Sjögren's syndrome in children for the same reasons we are adding a Sjögren's syndrome listing for adults in part A.

Other Changes

We are making minor conforming changes in prior 1.00B and 101.00B, and 1.00L and 101.00L to reflect changes in the final immune body system listings.

We are also making minor conforming changes in prior 8.00D3 and 108.00D3 of the skin disorders listings. We are revising these sections to indicate that we evaluate Sjögren's syndrome under the new listing for that disorder, final listings 14.10 and 114.10.

We are also making minor conforming changes in prior 13.00A and 113.00A of the malignant neoplastic diseases listings. We are revising these sections to reflect changes in final listings 14.08E and 114.08E.

Throughout these final rules, we are also making a number of minor editorial changes from the NPRM that we have not summarized above. For example, we have corrected unintentional language inconsistencies between part A and part B, changed sentences to use active voice instead of passive voice, and removed some repetitive statements and unnecessary words. None of these revisions are substantive, and they do not change the meaning of what we originally proposed in the NPRM.

Public Comments on the NPRM

In the NPRM, we published in the

Federal Register

on August 04, 2006 (71 FR 44432, corrected at 71 FR 46983), we provided the public with a 60-day comment period that ended on October 13, 2006. In addition to our notice to the public, we invited comments from national medical organizations and professionals, advocacy groups, and legal services organizations.

We received 55 comment letters. The commenters included advocacy groups, legal services organizations, State agencies that make disability determinations for us, medical organizations, and individuals, including individuals who have immune system disorders or relatives with immune system disorders. One of the comment letters reflected the comments from 40 organizations. We carefully considered all of the comments and provide our reasons for adopting or not adopting the comments in our responses below. Because some of the comments were long, we have condensed, summarized, and paraphrased them. We believe we have presented the commenters' views accurately, and have responded to all of the significant issues raised by the commenters that were within the scope of these rules.

Some commenters also wrote in about issues that were not related to the proposed rules, and in some cases not to Social Security disability benefits. Although we did read those letters, we did not respond to them.

Also, some commenters sent comments supporting the rules changes and noting provisions with which they agreed without suggestions for changes in those provisions. In most cases, we have not summarized or responded to those comments below because they do not require a response. However, we appreciate receiving them.

Use of Functional Criteria in the Immune System Disorders Listings

Comment:

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

these listings. The commenter observed that, while the consideration of functional impacts may result in greater latitude among adjudicators and more flexibility in decisionmaking, there is also an element of subjectivity that could result in greater inconsistency in our decisions. The second commenter, who generally agreed that “functioning should be considered in ratings,” said that the addition of functional criteria to the listings for immune system disorders other than HIV infection would not make the evaluation of these disorders any easier. This commenter said that considering functional information in claimant and third party reports of activities of daily living, and treating physician and other source statements would make evaluating these disorders more difficult. The commenter also believed that more evidence would be needed to support the decisions.

We address the third commenter's concern in the next comment and response.

Response:

As we explained in the NPRM (71 FR at 44440) and earlier in this preamble, we are adding the functional criteria in response to many comments we received on the ANPRM and in public outreach meetings. As many commenters pointed out, the debilitating effects of immune system disorders are often “invisible”; that is, outward signs of the disorders and objective severity markers often are not obvious and we cannot describe them in a listing. Because of this, the proposal received support from many individuals (or their family members) who received disability benefits only after going through a long appeals process. We also received comments about inconsistencies in our adjudications because we did not provide the kinds of guidance about evaluating the functional impact of immune system disorders that we do in these final rules.

Therefore, we do not agree with the commenters who thought that adding the functional criteria would have the negative effects they described or that we should not add functional criteria to these listings. To the contrary, we believe that these final listings will result in more consistent adjudications, and in some cases, faster adjudications, a need for less development, and fewer cases in which appeals are necessary, as we explain in more detail below.

The final listings describe individuals who are very ill. To qualify under one of these listings, an individual must first establish with objective medical evidence that he or she has the type of immune system disorder described by a given listing. Second, the individual must show that he or she repeatedly becomes ill as a result of the impairment. These two findings alone establish that the individual has a significant medical problem. The third requirement, to show a “marked” limitation in at least one of the areas of functioning, establishes that the overall impairment causes serious limitations.

A “marked” limitation as we define it is an obvious, serious limitation that affects all aspects of the individual's life (activities of daily living, social functioning) or the ability to do tasks (deficiencies in concentration, persistence, or pace). Therefore, it can be easier for an adjudicator to assess whether there is a “marked” limitation in an area of functioning, and to justify that assessment, than it is to assess and justify a residual functional capacity assessment. Residual functional capacity is more detailed, requiring evaluation of specific physical and mental work-related functions, what we often call a “function-by-function” assessment.

Because of this, without these final listings, our adjudicators would have to do more work in most, if not all, cases of individuals who have immune system disorders that will meet these final listings only to reach the same decision. Under the prior rules, virtually all of the individuals who could now qualify under the new functional listings required a residual functional capacity assessment. Our adjudicators not only had to do additional work to provide this more detailed assessment of functioning, but they also had to do the additional work associated with making findings about the ability to do past relevant work at step 4 of the sequential evaluation process, and to make an adjustment to other work at step 5. Each of these determinations—function-by-function residual functional capacity assessment, assessment of the ability to do past relevant work, and ability to make an adjustment to other work—required development of information. We believe that in some cases adjudications under these final listings will be easier, faster, and more consistent.

Finally, we have significant experience applying these and similar functional criteria in many claims. We began using these functional criteria in listing 14.08 in 1993. We used some of the same criteria to evaluate physical impairments in children when we first implemented the policy of functional equivalence for children in 1991,

2

and have used similar kinds of criteria for evaluating functional equivalence in physical impairment claims since 2000 under § 416.926a of our rules (65 FR 54747 (2000)). Many of our listings, including most of our musculoskeletal listings, several of our cardiovascular listings, and most of the neurological listings, contain functional criteria.

2

See generally 56 FR 5534 (1991).

Comment:

The third commenter (whose comment was about the functional criteria in proposed listing 14.08) suggested that limitations in maintaining social functioning and in completing tasks in a timely manner due to deficiencies in concentration, persistence, or pace are basic issues for evaluating mental impairments under 12.00, for mental disorders, and should be removed from the listing. Similarly, one of the two commenters whose comments we summarized in the preceding comment summary expressed concern that adjudicators could assume that the functional criteria in listing 14.08 pertain only to the evaluation of mental impairments because they are similar to those considered in the context of the mental listings.

Response:

We do not agree that maintaining social functioning or completing tasks in a timely manner due to deficiencies in concentration, persistence, or pace describe only mental functioning and should be removed from listing 14.08K or any of the other corresponding final listings. We addressed this issue at length in 1993 when we first published these rules. In the preamble to the 1993 publication of the rules, we explained in responding to public comments:

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

58 FR at 36040. We also explained that we had modified the language of the introductory text to make it more specific to individuals with HIV infection. Those modifications remain in these final rules with even further clarifications.

A number of commenters on the 1993 rules specifically commented that the area of social functioning is meant to measure an individual's psychiatric

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

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

58 FR at 36041.

In addition, and as we noted in the response immediately preceding this one, over the almost 15 years since we first published listing 14.08, we have gained considerable experience applying functional criteria such as these to physical impairments.

In final 14.00I, as in the NPRM, we provide that functional limitations may result from the impact of the disorder on mental functioning, physical functioning, or both mental and physical functioning. As we indicated in the NPRM, we revised 14.00I so that it applies to all of the listed impairments and more consistently refers to symptoms that are related to physical impairments. We believe that these revisions will help our adjudicators to better understand and remember that the areas of functioning should be applied to physical, as well as mental, limitations. However, we will provide training on the new functional criteria in these final rules.

Comment:

One commenter said that adjudicators will need guidance on how to determine whether to use the immune system disorders listings alone versus completing the typical full documentation required for the mental disorders listings. The commenter remarked that doing additional mental development such as obtaining a consultative examination for a mental status examination could potentially delay a claimant's determination.

Response:

We agree that guidance is needed and plan to address this issue in the training that we will conduct on these final rules. We do not believe that mental consultative examinations will be required as a result of these final listings because we are not trying to document mental impairments. Rather, we are determining any functional limitations and restrictions that a person may have as a result of his or her immune system disorder(s). As we do for other impairments, such as HIV infection, we would expect adjudicators and reviewers to assess functioning by evaluating objective medical evidence and evidence from other sources as described in §§ 404.1512 and 416.912.

Comment:

One commenter suggested that we provide more concrete guidance on how to evaluate the severity of limitations in activities of daily living and more structure on the application of terms such as “moderate, marked, and extreme” to reduce the likelihood of inconsistent interpretation of these terms.

Response:

We did not adopt this comment because the application of these terms is often dependent on specific case facts, and because we believe that any additional detail would be better presented in training and other instructions. Our adjudicators have considerable experience evaluating “marked” and “extreme” limitations and have used the functional criteria in prior listing 14.08N which are similar to the criteria we include in these final rules. However, we will remind adjudicators of our guidance in these areas when we conduct training on these final rules.

Comment:

One commenter referred to proposed 14.00I and said that it “introduce[d] the concept of ‘repeated manifestations accompanied by functional limitations' ” and the application of this concept to eight listings. The commenter observed that this “new way of evaluating the impact of repetitive episodes” was “sound in theory” but “may be difficult to apply in practice” because of the implicit need to document activities of daily living during periods sometimes well in the past. The commenter suggested that we clarify that the intent of the listings that include standards for evaluating functional limitations resulting from repeated manifestations of immune system disorders is to document functional limitations occurring in the present and does not require extensive documentation of the impact on activities of daily living during earlier episodes. The commenter indicated that evaluating the impact of repetitive episodes may be difficult because of the extended time period for which we may need to develop documentation of activities of daily living.

Response:

We believe we accommodated this comment by adding language in final 14.00I3 explaining that the manifestation episodes must occur within the period covered by the claim. As we already do, for example, whenever we need to assess residual functional capacity, we will develop evidence about the individual's functioning for the entire period covered by the claim. The final rules do not impose any additional burden in that regard, as we have explained in our responses to the preceding comments.

Also, we must note that the concept of repeated manifestations accompanied by functional limitations is not new. We have used the criterion in the HIV infection listings since 1993. The innovation in these final rules is to apply the same kind of criterion to the other listed immune system disorders.

Systemic Lupus Erythematosus (SLE)

Comment:

One commenter thought that the terms “repeated,” “marked,” and “manifestation” in the SLE listing could cause confusion for physicians and adjudicators. The commenter recommended that we clarify the definition of each term or replace the section in the SLE listing with a different rule, which the commenter also proposed. (We address the proposal to replace the SLE listing in a later comment and response.)

With regard to the term “marked,” the commenter believed that our proposed definition was ambiguous. The commenter suggested that we add more examples of “marked” and define it, giving examples of “moderate” for comparison. The commenter also said that physicians do not use the term “marked” in describing limitations resulting from SLE.

The commenter also suggested that we provide a definition of “manifestation” with examples because it was not defined in the proposed rule.

Response:

We do not expect physicians and other medical sources to use our terminology. We only need for them to provide us with medical evidence that we will use to determine whether an individual's impairment meets the requirements of a listing. For example, a physician does not need to tell us that a flare of his or her patient's SLE was a “manifestation,” only report to us what occurred in medical terms, and if necessary, provide an opinion that it was related to the SLE.

Likewise, we realize that physicians may not use the term “marked” in describing limitations resulting from SLE. However, for the purpose of determining disability, the issue of whether an individual has a “marked” limitation is an administrative finding that we make based upon consideration of all relevant evidence in the individual's case record, which may include information that the treating source does not have. We only need evidence describing the individual's limitations, and we will determine whether those limitations meet our definition of “marked.”

The definitions of the terms “repeated” and “marked” in these final rules are substantively the same as the definitions of these terms in our prior rules, and our adjudicators have been using these definitions since 1993, when we issued the prior rules. As we have already noted, we use the term “marked” in a number of our other rules as well.

Comment:

With regard to the term “repeated,” the same commenter indicated that patients might not see their physicians often enough to satisfy the criterion in the proposed rule, or physicians might not record the required information in a patient's chart. The commenter said that physicians may not spend time documenting their records because of time constraints, and this would be a problem if the individual later applies for disability benefits.

Response:

We understand the commenter's concern. However, such individuals with SLE can still qualify under final listing 14.02A, which does not require a showing of repeated manifestations, and in other ways; for example, with impairment manifestations that meet other listings, based on our policy of “medical equivalence,” or based on residual functional capacity. We address the latter issues in final 14.00G6 for individuals who have not received ongoing treatment or do not have an ongoing relationship with the medical community, and final 14.00J, for individuals whose impairments do not meet the requirements of one of these listings.

Comment:

The commenter also said that the requirement for repeated manifestations did not recognize that SLE can cause permanent damage that remains chronic after the manifestations have stopped. As an example, the commenter described an individual who had a severe heart attack caused by lupus, who does not experience any new manifestations, but who is disabled from permanent heart damage.

Response:

The example of an individual who has permanent, disabling heart damage that the commenter provided is an example of the principles we discussed in the response immediately above. If the heart damage is sufficiently severe, it would meet or medically equal one of our cardiac listings in 4.00, the cardiovascular body system. Even if it does not meet or medically equal a listing in the cardiovascular body system, it could be the basis for a finding of disability at the last step of the sequential evaluation process because of the functional limitations it causes.

Also, our criteria for evaluating repeated manifestations of SLE do not require repetition of the same manifestation. For example, an individual who has experienced three different manifestations of SLE (for example, heart problems, leukopenia, and pleuritis) with the frequency and duration required in final 14.00I3 would have an impairment that satisfies the criterion in final listing 14.02B. In response to this comment, we have added language to final 14.00I3 to make this clear. This is not a change in what we proposed, only a clarification of our intent.

Comment:

The same commenter also suggested that we use the term “flare” instead of “manifestation” because that is the word physicians treating SLE use to describe increased symptoms and disease activity.

Response:

We are aware that physicians who treat SLE often use the term “flare” to describe increased symptoms and disease activity. However, “flare” implies a temporary state, and our term “manifestation” does not necessarily mean that. We believe that many medical professionals who do not work for us will understand our term, but it is not critical that they do.

Comment:

The same commenter provided a suggested replacement for the criteria in proposed listing 14.02B that included language such as “severe impairment” in one of the domains and the “opinion” of a specialist regarding prognosis for improvement in functional capacity. The commenter indicated that the proposed criteria were medically accurate for evaluating lupus, could be documented through a claimant's medical records, and could be easily applied by adjudicators.

Response:

We did not adopt the recommendation for a number of reasons. The commenter's criteria included essentially the same criteria we had proposed. However, the commenter would have also required medical evidence that shows that treatment has not significantly reduced the severity of the disorder and is not likely to restore the capacity to work. This would have made the listing stricter than what we had proposed and stricter than the prior listing.

Comment:

One commenter suggested that we add “intense generalized muscle aches and pains” to the constitutional symptoms and signs of severe fatigue, fever, malaise, or weight loss in proposed listing 14.02 because it is the most common symptom that rheumatologists who treat individuals with lupus hear from their patients.

Response:

We agree that intense generalized muscle aches and pains is a common complaint of individuals with SLE. However, these symptoms generally respond to treatment. If the muscle aches and pains persist or do not respond to treatment, they may be the result of a secondary disorder other than SLE. Therefore, we did not adopt this comment.

Systemic Sclerosis (Scleroderma)

Comment:

One commenter suggested that we should make the criterion for toe contractures in listing 14.04B1 more specific to make it more comparable with the criteria for finger contractures in proposed listing 14.04B2, atrophy of the lower extremities in proposed listing 14.04B3, and atrophy of the upper extremities in proposed listing 14.04B4. The commenter remarked that ordinary hammer toes are contractures and o

This text is long and has been trimmed here. Open the source document for the complete record.