Under title XVI of the Act, we provide for Supplemental Security Income (SSI) payments on the basis of disability if you are disabled and have limited income and resources.

How do we define disability?

Under both the title II and title XVI programs, disability must be the result of any medically determinable physical or mental impairment or combination of impairments that is expected to result in death or which has lasted or is expected to last for a continuous period of at least 12 months. Our definitions of disability are shown in the following table:
Disability means you have a medically determinable impairment(s) as described above that If you file a claim under * * * And you are * * * results in * * * Title II............................ an adult or a child.... the inability to do any substantial gainful activity (SGA). Title XVI........................... a person age 18 or the inability to do any SGA. older.
Title XVI........................... a person under age 18.. marked and severe functional limitations. What are the listings?

The listings are examples of impairments that we consider severe enough to prevent you as an adult from doing any gainful activity. If you are a child seeking SSI payments based on disability, the listings describe impairments that we consider severe enough to result in ``marked and severe functional limitations.'' Although we publish the listings only in appendix 1 to subpart P of part 404 of our rules, we incorporate them by reference in the SSI program in Sec. 416.925 of our regulations, and apply them to claims under both title II and title XVI of the Act.

How do we use the listings?

The listings are in two parts. There are listings for adults (part A) and for children (part B). If you are a person age 18 or over, we apply the listings in part A when we assess your claim, and we never use the listings in part B.

If you are a person under age 18, we first use the criteria in part B of the listings. If the listings in part B do not apply, and if the specific disease process(es) has a similar effect on adults and children, we then use the criteria in part A. (See Sec. Sec. 404.1525 and 416.925.)

If your impairment(s) does not meet any listing, we will also consider whether it medically equals any listing; that is, whether it is as medically severe. (See Sec. Sec. 404.1526 and 416.926.)

We use the listings only to decide that you are disabled or that you are still disabled. We will never deny your claim or decide that you no longer qualify for benefits because your impairment(s) does not meet or medically equal a listing. If you have a severe impairment(s) that does not meet or medically equal any listing, we may still find you disabled based on other rules in the ``sequential evaluation process'' that we use to evaluate all disability claims. (See Sec. Sec. 404.1520, 416.920, and 416.924.)

Also, when we conduct reviews to determine whether your disability continues, we will not find that your disability has ended based only on any changes in the listings. Our regulations explain that, when we change our listings, we continue to use our prior listings when we review your case, if you qualified for disability benefits or SSI payments based on our determination or decision that your impairment(s) met or medically equaled the listings. In these cases, we determine whether you have experienced medical improvement and,
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if so, whether the medical improvement is related to the ability to work. If your condition(s) has medically improved so that you no longer meet or medically equal the prior listing, we evaluate your case further to determine whether you are currently disabled. We may find that you are currently disabled, depending on the full circumstances of your case. See Sec. Sec. 404.1594(c)(3)(i) and 416.994(b)(2)(iv)(A). If you are a child who is eligible for SSI payments, we follow a similar rule after we decide that you have experienced medical improvement in your condition(s). See Sec. 416.994a(b)(2). Why are we proposing to revise the listings for immune system disorders?

We are proposing these revisions to update the listings and to provide more information about how we evaluate immune system disorders. We have not updated these rules since we first published them in 1993 (58 FR 36008). At that time, we established body system listings for immune system disorders in part A and part B. We made those rules effective for 5 years from the date of publication, unless we extended them, or revised and issued them again (58 FR at 36051). Since that time, we have extended the expiration date of the immune body system listings but we have not comprehensively revised them.

We have, however, made several changes to these listings over the years. On November 19, 2001, we also published final rules in the Federal Register adding listings 14.09 and 114.09, for inflammatory arthritis, to these body system listings, including introductory text to those listings in sections 14.00B6 and 114.00E (66 FR 58009). We published minor technical changes to these body system listings on February 24, 2002 (67 FR 20018).

How did we develop these proposed rules?

These proposed rules reflect our adjudicative experience and advances in medical knowledge, treatment, and methods of evaluating immune system disorders. They also reflect comments we asked you to provide to help us develop the proposals.

We published an Advance Notice of Proposed Rulemaking (ANPRM) in the Federal Register on May 9, 2003 (68 FR 24896). The purpose of the ANPRM was to inform the public that we were planning to update and revise the rules we use to evaluate immune system disorders and to invite interested individuals and organizations to send us comments and suggestions for updating and revising the immune system listings. In the ANPRM, we provided a 60day period for comments and suggestions; that period ended on July 8, 2003. We received over 200 letters and e mails in response to the notice, many from individuals who have immune system disorders or who have family members with such disorders. We also received comments from medical experts, advocates, and people who adjudicate claims for us. Although we are not summarizing or responding to the comments in this notice, we read and considered them carefully and are proposing changes in our rules based on some of the suggestions we received.

We also hosted policy conferences on ``Immune System Disorders in the Disability Programs'' in Philadelphia, PA, on December 15, 2003, and in San Francisco, CA, on February 18 and 19, 2004. At these conferences, we heard comments and suggestions for updating and revising these rules from individuals who have immune system disorders and their family members, physicians who treat individuals with immune system disorders, other professionals who work with people who have immune system disorders, advocates who represent individuals with immune system disorders, and individuals who make disability determinations and decisions for us in the State agencies and the Office of Hearings and Appeals. Several of the changes we propose in these rules are based on information we obtained at these conferences. When will we start to use these rules?

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

How long would these proposed rules be effective?

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

We are proposing to:

We propose to expand and reorganize the introductory text to these listings. There are four major sections in current 14.00, and the longest of those sections, 14.00D, addresses only the evaluation of HIV infection. In these proposed rules, we add more sections and expand the guidance we provide about evaluating other kinds of immune system disorders.

Some of the guidance in current 14.00D is useful for evaluating other kinds of immune system disorders in addition to HIV infection. We are proposing to move that guidance from current 14.00D to new sections that would have more general applicability to immune system disorders. We are not proposing to remove any substantive guidance about how we evaluate HIV infection, only to reorganize some of the
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information now in 14.00D of the current rules and to give it broader applicability where appropriate. We are also proposing to update and expand some of the guidance we provide for evaluating HIV infection and its effects, as we describe in more detail below.

The four sections in the current rules are:

In the proposed rules, there are 10 sections in the introductory text. The first three sections (proposed 14.00A, B, and C) provide general information about this body system, including definitions of terms. Each of the next three sections describes a particular category or type of immune system disorder: Autoimmune disorders (proposed 14.00D); immune deficiency disorders, excluding HIV infection (proposed 14.00E); and HIV infection (proposed 14.00F). The next three sections explain how we consider the effects of your treatment (proposed 14.00G), your symptoms (proposed 14.00H), and the functional limitations from your immune system disorder under these listings (proposed 14.00I). The last section, proposed section 14.00J, explains how we consider the effects of your immune system disorder when it does not meet the requirements of one of the proposed immune system listings. We are designating all paragraphs in the proposed rules with letters or numbers to make it easier to refer to them. We are also providing headings for all of the major sections and many of the subsections.

The following are the names of the major sections in proposed 14.00. We describe each section in detail later in this preamble.

The following is a detailed description of the proposed changes in the introductory text of these proposed rules.

14.00 Immune System Disorders

We propose to change the name of this body system from ``Immune System'' to ``Immune System Disorders'' to more accurately reflect that we use these listings to evaluate immune system disorders in accordance with the requirements of the disability program.
Proposed 14.00AWhat disorders do we evaluate under the immune system listings?

In proposed 14.00A, we provide a brief overview of this body system. We explain the kinds of disorders we evaluate under the immune system listings and that we organize these impairments under the categories of ``autoimmune disorders,'' ``immune deficiency disorders, excluding HIV infection,'' and ``HIV infection.'' Proposed 14.00A has four subsections.

We incorporate current 14.00A in the opening sentence of proposed 14.00A1. We propose to revise the sentence, which explains the kinds of immune system dysfunction that immune system disorders may cause, to update and simplify it. In proposed 14.00A1a and 14.00A1b, we incorporate the first sentence in the sixth paragraph of current 14.00B to explain that immune system disorders can cause dysfunction in one or more components of the immune system, and describe ways in which immune system disorders may result in loss of function. In the second sentence of 14.001b, we propose to add ``involuntary'' as a descriptor of weight loss to clarify that we mean weight loss due to an immune system disorder(s) or its treatment. We are adding ``involuntary'' as a descriptor of weight loss throughout the introductory text in part A and part B for this same reason. Proposed 14.00A1c is a new paragraph that explains how we have organized immune system disorders in the preface (introductory text) of these listings.

In proposed 14.00A2, Autoimmune disorders, we incorporate the first paragraph in current 14.00B to provide a brief description of autoimmune disorders. We propose to add an explanation that these disorders are sometimes referred to as ``rheumatic diseases,'' ``connective tissue disorders,'' or ``collagen vascular disorders'' and that some of the features of these disorders in adults differ from the features of the same disorders in children. We provide a cross reference to proposed 14.00D, the section of the introductory text that addresses autoimmune disorders in detail. We also propose to remove the last sentence of the first paragraph of current 14.00B, which explains that connective tissue disorders generally evolve and persist over time, may result in functional loss, and may require longterm, repeated evaluation and management, because it does not provide useful adjudicative guidance. However, we do explain in proposed 14.00A1b that immune system disorders can cause limitation(s) that result in an ``extreme'' loss of function.

Proposed 14.00A3, Immune deficiency disorders, excluding HIV infection, is new. We explain that these disorders can be classified as ``primary'' or ``acquired,'' are characterized by recurrent or unusual infections, and are associated with an increased risk of malignancies and of other autoimmune disorders. We also provide a crossreference to proposed 14.00E, the introductory section that addresses immune deficiency disorders in detail.

In proposed 14.00A4, Human immunodeficiency virus (HIV) infection, we provide a brief description of HIV infection. We propose to move the first sentence in current 14.00D1 to this section. The sentence explains that HIV infection is caused by a specific retrovirus and may be characterized by increased susceptibility to opportunistic infections, cancers, or other conditions.
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We also provide a crossreference to proposed 14.00F, the section of the introductory text that addresses HIV infection in detail. Proposed 14.00BWhat information do we need to show that you have an immune system disorder?

In proposed 14.00B, we incorporate the first sentence of the second paragraph of current 14.00B to explain what information we need to show that you have an immune system disorder. We moved the second and third sentences of the second paragraph of current 14.00B, which define our term ``appropriate medically acceptable imaging,'' to proposed 14.00C, a new section that provides definitions of terms in these listings. We propose to remove the last two sentences of the current paragraph. They explain that we will not purchase tests that may involve significant risk; however, we already include this general policy in Sec. Sec. 404.1519m and 416.919m of our regulations so it is not necessary to repeat them in this section.

In the second sentence of proposed 14.00B, we provide that ``we will make every reasonable effort'' to obtain your medical history, medical findings, and the results of laboratory tests in documenting whether you have an immune system disorder. We include this requirement in current 14.00D, for HIV infection, but we do not include similar guidance in current 14.00B, for connective tissue disorders. We propose to add this guidance under proposed 14.00B because it is appropriate for all immune system disorders.

We also propose to remove the third and fourth paragraphs of current 14.00B. The third paragraph of current 14.00B provides that we need a longitudinal clinical record of at least 3 months demonstrating active disease to assess the severity and duration of your impairment. However, this is not always the case, even under the current rules. For example, individuals with HIV infection and cryptococcal meningitis (current listing 14.08B4) or Kaposi's sarcoma (current listing 14.08B8), and individuals with ankylosing spondylitis with fixation (ankylosis) of the dorsolumbar spine at 45[deg] (current listing 14.09B2) are disabled based on those findings alone. In that case, we do not need 3 months of evidence or evidence showing active disease. Other cases may be decided with less than 3 months of evidence, while others may require more than 3 months of evidence. Therefore, we are removing this guidance because each case should be decided on an individual basis.

Proposed 14.00CDefinitions

In proposed 14.00C, we define what we mean by important terms in these listings. As already noted, we include the definition of ``appropriate medically acceptable imaging'' from the second paragraph of current 14.00B. However, we propose to replace the word ``proper'' in the second sentence of this definition with the phrase ``generally accepted and consistent with the prevailing state of medical knowledge and clinical practice'' to more clearly explain what we mean. We also propose to include in this new section the definitions of the terms ``severe'' from the sixth paragraph of current 14.00B, ``inability to ambulate effectively'' and ``inability to perform fine and gross movements effectively'' from current 14.00B6b, and ``resistant to treatment,'' ``recurrent,'' and ``disseminated'' from the second, third, and fourth paragraphs of current 14.00D2. All of these terms will apply to several, and sometimes all, of the proposed listings in this body system.

In proposed 14.00C, we do not include the phrase ``must have lasted, or be expected to last, for at least 12 months'' from the definitions of ``inability to ambulate effectively'' and ``inability to perform fine and gross movements effectively'' in current 14.00B6b because we believe it is unnecessary. Unless an impairment is expected to result in death, it must have lasted or must be expected to last for a continuous period of at least 12 months to meet the definition of disability. This proposed change would also make the definitions of the terms consistent with the definitions of the same terms in 1.00B2b and 1.00B2c in the musculoskeletal body system.

We also propose to move and simplify the definitions of the terms ``resistant to treatment,'' ``recurrent,'' and ``disseminated'' in current 14.00D2, primarily to remove language that we believe is unnecessary. For example, we removed the explanation that the terms ``have the same general meaning as used by the medical community.'' These changes are only editorial. We do not intend the proposed definitions to be substantively different from the current rules.

In proposed 14.00C8, we reference current 1.00F for the definition of ``major peripheral joints'' instead of restating the definition as we do in current 14.00B6a. We also propose to add the definitions of several other important terms in these listings, including in proposed 14.00C2, the term ``constitutional symptoms or signs.'' In proposed 14.00C2, we also provide brief definitions for the constitutional symptoms ``severe fatigue'' and ``malaise.'' We propose to add these definitions in response to the many comments we received that indicated that the fatigue and malaise that people who have immune system disorders experience can be very limiting.
Proposed 14.00DWhat are the listed autoimmune disorders in these listings?

In proposed 14.00D, we incorporate and expand upon the information in current 14.00B1 through 14.00B6, which describe features commonly associated with each of the listed autoimmune system disorders. Throughout these sections, we refer to ``autoimmune disorders'' instead of ``connective tissue disorders'' because the phrase ``autoimmune disorders'' is more medically accurate and more frequently used. We also propose to add a new section 14.00D7 for Sj[ouml]gren's syndrome because we are proposing to add new listing 14.10 for that autoimmune disorder.

In proposed 14.00D1, Systemic lupus erythematosus (14.02), we expand and clarify the information in current 14.00B1. In proposed 14.00D1a, General, we explain that systemic lupus erythematosus (SLE) may involve any organ or body system and describe by body system some potential manifestations that may be involved. We expand our explanation of how SLE is frequently characterized clinically and propose to change ``fatigability'' used in current 14.00B1 to ``fatigue'' to be consistent with how we describe this symptom throughout the immune system listings. We also add ``involuntary'' as a descriptor of weight loss to clarify that we mean weight loss due to SLE or its treatment. In proposed 14.00D1b, Documentation of SLE, we propose to update our rules to explain that your medical evidence will generally, but not always, show that your SLE satisfies the criteria in the ``Criteria for the Classification of Systemic Lupus Erythematosus'' by the American College of Rheumatology, found in the most recent edition of the Primer on the Rheumatic Diseases published by the Arthritis Foundation. This is a more uptodate reference than the 1982 reference in the current rules.

In proposed 14.00D2, Systemic vasculitis (14.03), we clarify the information in the current rule. Proposed 14.00D2a, General, corresponds to the first three sentences of current 14.00B2. In it, we explain that vasculitis is an inflammation of blood vessels that may occur acutely in association with adverse drug reactions, certain chronic infections, and
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occasionally malignancies, and that it may also be associated with other autoimmune disorders. We also give examples of several clinical patterns in which it may occur. We propose to remove the fourth sentence of current 14.00B2, which describes cutaneous vasculitis, because the impairment varies greatly in its manifestation, may not be associated with systemic involvement, and would not be expected to result in a listinglevel impairment.

Proposed 14.00D2b, Documentation of systemic vasculitis, corresponds to the last two sentences of current 14.00B2. In it, we describe documentation that we use to confirm the diagnosis of systemic vasculitis.

Proposed 14.00D3, Systemic sclerosis (scleroderma) (14.04), corresponds to current 14.00B3. We propose to revise the heading and to expand the information in the section. Proposed 14.00D3a, General, corresponds to the first three sentences of current 14.00B3. We propose to change the term ``Raynaud's phenomena,'' which we use in the second and third sentences of current 14.00B3, to ``Raynaud's phenomenon'' because the latter is the correct term. We make this same change in proposed listing 14.04C. In proposed 14.00D3b, Diffuse cutaneous systemic sclerosis, we continue to explain that, in addition to skin or blood vessels, major organ or systemic involvement may include the gastrointestinal tract, lungs, heart, kidneys, and muscle. This guidance corresponds to the fourth sentence in the current rule.

Proposed 14.00D3c, Localized scleroderma (linear scleroderma or morphea), is new. We propose to add this section and appropriate listings in proposed 14.04 for these disorders that originate in childhood because their disabling effects can persist into adulthood. Proposed 14.00D3c is essentially the same as proposed 114.00D3c, which we describe in detail later in this preamble.

Proposed 14.00D3d, Documentation of systemic sclerosis (scleroderma), is also new. In it, we explain what documenting systemic sclerosis (scleroderma) involves and that there may be an overlap with other autoimmune disorders.

In proposed 14.00D4, Polymyositis and dermatomyositis (14.05), we clarify the information in current 14.00B4. Proposed 14.00D4a, General, corresponds to the first three sentences of current 14.00B4. It describes the characteristics of polymyositis and dermatomyositis. In proposed 14.00D4b, Documentation of polymyositis or dermatomyositis, we describe the findings that are generally used to document these impairments. The first sentence of the proposed rule corresponds to the last sentence of current 14.00B4. We propose minor editorial revisions, including the removal of the reference to ``myositis'' because there are multiple characteristic abnormalities on muscle biopsy that support the diagnosis of polymyositis or dermatomyositis. We also propose to add a sentence to explain that people with dermatomyositis have a characteristic skin rash.

In proposed 14.00D4c, Additional information about how we evaluate polymyositis and dermatomyositis under the listings, we explain how we evaluate commonly occurring limitations associated with these disorders. Proposed 14.00D4c(i) corresponds to the fourth and fifth sentences of current 14.00B4. We propose to delete the example of weakness of the anterior neck flexor muscles in the sixth sentence of current 14.00B4 because we are proposing to delete the reference to the cervical muscles from listing 14.05 for reasons we explain later in this preamble. We also propose to add an example of squatting. Squatting is a common means for evaluating weakness in the pelvic girdle muscles.

In proposed 14.00D4c(ii), we explain that we will evaluate malignancies (which may be associated with these disorders) under the malignant neoplastic diseases listings (13.00ff). We do not provide this guidance in proposed 114.00D4c in the childhood section for polymyositis or dermatomyositis because malignancies are not commonly associated with these disorders in children. We also explain that we evaluate the involvement of other organs or body systems under the affected body system.

In proposed 14.00D5, Undifferentiated and mixed connective tissue disease (14.06), we reorganize and clarify the information in current 14.00B5. In the proposed rules, we are adding an explicit reference to mixed connective tissue disease (MCTD) to clarify what we mean in the current rules when we refer to ``overlap'' syndromes. This is not a substantive change, but a clarification of our current rules to update medical terminology. In proposed 14.00D5a, General, we describe what we mean by undifferentiated and mixed connective tissue disease. In proposed 14.00D5b, Documentation of undifferentiated and mixed connective tissue disease, we explain when clinical features and serologic findings may be used to diagnose undifferentiated and mixed connective tissue disease. These provisions in proposed 14.00D5a and 14.00D5b are not substantively different from the provisions in the first three sentences of current 14.00B5.

We propose to delete the last sentence of current 14.00B5. The current sentence indicates that the correct designation of an ``overlap'' disorder is important for the assessment of prognosis. We believe that this sentence, while useful in treatment settings, does not provide useful adjudicative guidance.

In proposed 14.00D6, Inflammatory arthritis (14.09), we expand, reorganize, and clarify the rules in current 14.00B6. Proposed 14.00D6a, General, corresponds to the first and fourth sentences of current 14.00B6. We continue to explain that inflammatory arthritides include a vast array of disorders that differ in cause, course, and outcome and may result in difficulties of ambulation or fine and gross movements. We edited the fourth sentence of current 14.00B6 to break it up into three shorter sentences. However, we do not intend to change the meaning of the provision.

Proposed 14.00D6b, Inflammatory arthritides involving the axial spine (spondyloarthropathies), and 14.00D6c, Inflammatory arthritides involving the peripheral joints, correspond to the second and third sentences of current 14.00B6. In these sections, we list some disorders that may be associated with inflammatory spondyloarthropathies involving the axial spine (proposed 14.00D6b) and inflammatory arthritides affecting the peripheral joints (proposed 14.00D6c). We propose to add inflammatory bowel disease (IBD) to the lists of examples in both sections because arthritis is the most common extra intestinal complication of IBD. In proposed 14.00D6b, we remove the examples of ``other reactive arthropathies'' and ``undifferentiated spondylitis'' now included in the second sentence of current 14.00D6 because they are nonspecific and the list is not intended to be complete, only to provide some examples. Finally, we propose to update some of the terminology in this section; for example, we refer to ``psoriatic arthritis'' instead of ``psoriatic arthropathy.''

Proposed 14.00D6d, Documentation of inflammatory arthritides, is new. In it, we explain that generally, but not always, the diagnosis of inflammatory arthritis is made by the clinical features and serologic findings described in the most recent edition of the Primer on the Rheumatic Diseases.

Proposed 14.00D6e, How we evaluate the inflammatory arthritides under the
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listings, corresponds to the information in the last two sentences of current 14.00B6, current 14.00B6c, and current 14.00B6d. We are reorganizing the text to reflect the proposed reorganization of listing 14.09, which we explain later in this preamble, and to clarify it.

We are not including the provisions of current 14.00B6e in proposed 14.00D6. Current 14.00B6e provides that the fact that an individual is dependent on steroids, or any other drug, for the control of inflammatory arthritis is insufficient in itself to establish disability. We added it to part A of our listings in 2002 for consistency with 114.00E6, a provision we added to part B of the listings at the same time (66 FR 58010, 58020 (2001)). We are proposing to remove that provision for reasons we explain below in our summary of the proposed rules in part B. Therefore, we are proposing to remove this provision in part A for consistency with that change. However, in proposed 14.00G3, we continue to state that we will consider the adverse side effects of treatment, including the adverse effects of corticosteroids, to ensure that our adjudicators remember to consider the side effects an individual might experience from steroids and any other treatment.

Proposed 14.00D7, Sj[ouml]gren's syndrome (14.10), is new. As already noted, we are proposing to add a new listing for Sj[ouml]gren's syndrome. In connection with that proposed listing, proposed 14.00D7a, General, explains the features of the disorder, including its resulting symptoms and possible complications. We also list organ systems that may be involved and note that Sj[ouml]gren's syndrome may be associated with other autoimmune disorders. In proposed 14.00D7b, Documentation of Sj[ouml]gren's syndrome, we also explain that if you have
Sj[ouml]gren's syndrome, your medical evidence will generally, but not always, show that your disease satisfies the criteria in the ``Criteria for the Classification of Sj[ouml]gren's Syndrome'' found in the most recent edition of the Primer on the Rheumatic Diseases.
Proposed 14.00EHow do we evaluate immune deficiency disorders, excluding HIV infection (14.07)?

In proposed 14.00E, we add a new section describing how immune deficiency disorders (excluding HIV infection) are classified, documented, and evaluated. This section has four subsections.

In proposed 14.00E1, General, we explain that immune deficiency disorders are classified as either ``primary'' or ``acquired.'' Primary disorders are mainly seen in children but, due to recent advances in treatment, many affected children survive into adulthood.

In proposed 14.00E2, Documentation of immune deficiency disorders, we explain that documentation of these disorders may be made by laboratory evidence or by other generally acceptable methods consistent with the prevailing state of medical knowledge and clinical practice.

In proposed 14.00E3, Immune deficiency disorders treated by stem cell transplantation, we explain how we evaluate immune deficiency disorders that are treated in this way. In proposed 14.00E3a, Evaluation in the first 12 months, we explain that if you undergo stem cell transplantation we will consider you disabled until at least 12 months from the date of the transplant. This is the same provision that we use for most malignancies treated by bone marrow or stem cell transplants in the neoplastic listings. In 13.00L4 of those listings, we also included a special provision for autologous bone marrow transplantstransplants using your own stem cells (69 FR 67034). We do not include such an alternative provision in these proposed rules because people with immune deficiency disorders receive allogeneic transplantsthat is, stem cells taken from other people. Also, we propose to use ``stem cell transplantation'' instead of ``bone marrow or stem cell transplantation'' in this proposed section and in proposed listing 14.07B because ``stem cell transplantation'' is a broader term that encompasses different sites for obtaining hematopoetic (blood forming) stem cells, including bone marrow, peripheral blood, and umbilical cord blood. In proposed 14.00E3b, Evaluation after the 12 month period has elapsed, we explain that, after that period has elapsed, we consider any demonstrable residuals of your immune deficiency disorder including any residual impairment(s) resulting from your treatment. The provision also is based on 13.00L4 in our malignant neoplastic diseases listings.

Proposed 14.00E4, Medicationinduced immune suppression, is new. We explain that medication effects can result in immune suppression that will usually resolve once the medication is ceased. However, if you take prescribed medications for longterm 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.
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Proposed 14.00FHow do we evaluate human immunodeficiency virus (HIV) infection?

In proposed 14.00F, we incorporate, update, and expand information on HIV infection contained in current 14.00D3 through 14.00D7. We also make nonsubstantive editorial changes.

As already noted, we propose to move the first sentence of current 14.00D1 to proposed 14.00A4. Therefore, we begin proposed 14.00F with what is now the second sentence of current 14.00D1. It is a reminder that an individual with HIV infection need not meet the Centers for Disease Control definition of acquired immune deficiency syndrome (AIDS) to meet or medically equal the criteria of listing 14.08. We have made minor editorial changes to the sentence, but we do not intend to change its meaning.

We propose to move the provisions of current 14.00D2 to other sections in the proposed rules. In the first four paragraphs of current 14.00D2, we define the terms ``resistant to treatment,'' ``recurrent,'' and ``disseminated,'' and we would now define those terms in proposed 14.00C. In the fifth paragraph of current 14.00D2, we define ``significant involuntary weight loss'' for purposes of current listing 14.08I (which has become listing 14.08H in these proposed rules). In the proposed rules, we include this definition in 14.00F5.

Like current 14.00D3, proposed 14.00F1 is in two major sections: A section explaining how we document the diagnosis of HIV infection definitively (14.00F1a) and a section explaining how we document the diagnosis of HIV infection when we do not have definitive evidence (14.00F1b). In proposed 14.00F1, Documentation of HIV infection, we incorporate and update the information in current 14.00D3 to explain the laboratory tests or other evidence we accept as documentation of HIV infection. Proposed 14.00F1a, Documentation of HIV infection by definitive diagnosis, corresponds to current 14.00D3a. We propose to update and expand this section to include newer laboratory diagnostic techniques that did not exist or were not widely used when we published the current rules in 1993.

Proposed 14.00F1b, Other acceptable documentation of HIV infection, corresponds to current 14.00D3b. It explains what documentation of HIV infection we will accept instead of definitive laboratory testing. The proposed rule is essentially the same as the current rule except for nonsubstantive editorial changes.

In proposed 14.00F2, CD4 tests, we combine the provisions in the second undesignated paragraph after current 14.00D3a(iii) and the second paragraph in current 14.00D4a. We specify that, even though a reduced CD4 count or percent alone does not establish a definitive diagnosis of HIV infection, a CD4 count below 200/mm\3\ or 14 percent along with clinical findings does offer supportive evidence of opportunistic infections without a definitive diagnosis. This is because a CD4 count below 200 or 14 percent is an indicator of an increased susceptibility to developing opportunistic infections. We also make nonsubstantive editorial changes.

In proposed 14.00F3, Documentation of the manifestations of HIV infection, we incorporate the information in current 14.00D4 with nonsubstantive editorial changes. Like proposed 14.00F1 and current 14.00D4, proposed 14.00F3 is divided into two main parts. The first section explains how we document manifestation of HIV infection definitively (14.00F3a), and the second section explains how we document manifestations of HIV infection when we do not have definitive evidence (14.00F3b).

Proposed 14.00F3a, Documentation of the manifestations of HIV by definitive diagnosis, incorporates the first paragraph in current 14.00D4a.

In proposed 14.00F3b, Other acceptable documentation of the manifestations of HIV infection, we incorporate information that is in the first paragraph of current 14.00D4b. We propose to revise the language of this paragraph both editorially and to clarify our original intent. In the current rule, we indicate that ``if no definitive laboratory evidence is available, manifestations of HIV infection may be documented by medical history, clinical and laboratory findings, and diagnosis(es) indicated in the medical evidence.'' The sentence may imply that we need to have all of the things listed (medical history and clinical findings and laboratory findings and diagnosis(es)) to determine that you have a manifestation of HIV infection when we do not have definitive laboratory findings. That is not our intent, so we are clarifying in the proposed rule that we may need only some of this information to make a finding that you have a manifestation of HIV infection, depending on the prevailing state of medical knowledge and clinical practice. We also propose to clarify what we mean by ``laboratory findings'' in this context; that is, laboratory findings that do not in themselves definitively establish the existence of a diagnosis of an HIVrelated manifestation.

In 14.00D4 of the current rules we provide specific guidance for documenting one particular manifestation of HIV infection without definitive evidence: cytomegalovirus (CMV) disease. In proposed 14.00F3b, we expand the section to include two additional
manifestations. In proposed 14.00F3b(i), we add guidance to explain that Pneumocystis carinii pneumonia (PCP) is frequently diagnosed presumptively without definitive evidence and to provide examples of evidence that is supportive of a presumptive diagnosis of PCP. We also note that Pneumocystis carinii is now known as Pneumocystis jiroveci; however, ``PCP'' remains in common usage for the pneumonia caused by this organism.

In proposed 14.00F3b(ii), we incorporate and expand the information now in the second paragraph of current 14.00D4b, regarding the documentation of CMV disease. We propose to clarify that a positive serology test for CMV identifies a ``history'' of infection but does not confirm an ``active'' disease process. We do not include ``documentation of CMV disease requires confirmation by biopsy'' as in the last sentence of the second
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paragraph of current 14.00D4 because we are providing information on documentation other than definitive laboratory findings. Also, instead of stating that we can use generally acceptable methods to confirm the diagnosis of CMV, we provide examples of evidence, such as fever and positive CMV serology test, that are supportive evidence of a presumptive diagnosis of CMV disease.

In proposed 14.00F3b(iii), we add guidance on how toxoplasmosis of the brain is presumptively diagnosed since the definitive method of diagnosing toxoplasmosis of the brain by biopsy is not commonly performed.

In proposed 14.00F4, Manifestations specific to women, we incorporate the information in current 14.00D5. In proposed 14.00F4a, General, we incorporate the first paragraph of current 14.00D5 and in proposed 14.00F4b, Additional considerations for evaluating HIV infection in women, we incorporate the second paragraph of current 14.00D5. Except for adding paragraph designations and headings and minor editorial changes (including changes to reflect proposed changes in the paragraph designations of the listings explained below), the proposed provisions are the same as in the current rules.

In proposed 14.00F5, involuntary weight loss, we incorporate the last paragraph of current 14.00D2 with nonsubstantive editorial changes, including a change that reflects our proposal to redesignate listing 14.08I to listing 14.08H.
Proposed 14.00GHow will we consider the effect of treatment in evaluating your autoimmune disorder, immune deficiency disorder, or HIV infection?

In the current rules, we refer to treatment and its effects in four places.

We are proposing to remove the provisions in the third paragraph of 14.00B and paragraph 14.00B6e. Neither of those sections nor the other current rules we will continue to use contain provisions that explain in detail how we evaluate the positive effects and negative side effects of treatment in individuals who have autoimmune disorders and immune deficiency disorders apart from HIV infection. Also, most current treatments for HIV infection came into use, or came into wide use, after we first published listing 14.08 in 1993. As a consequence, we believe that current 14.00D7 needs to be updated to reflect the newer and more widely used treatments and treatment protocols for HIV infection and to reflect the considerable medical experience that has been gained since 1993 about the longterm effects, usefulness, and limitations of such treatments.

Therefore, we propose to add a new separate section 14.00GHow will we consider the effect of treatment in evaluating your autoimmune disorder, immune deficiency disorder, or HIV infection? The new section would address in one place issues of treatment that are common to all three types of immune system disorders as well as issues of treatment that are unique to each type of disorder, including treatment that is specifically for HIV infection. We do not propose to remove any guidance about treatment for HIV infection that is still relevant, only to move it to this new section. In fact, we propose to expand and update our rules to reflect what has been learned in applying different treatments for HIV infection since we published the current rules more than a decade ago. The provisions for addressing both the positive effects and negative side effects of treatment in individuals who have autoimmune disorders and immune deficiency disorders other than HIV infection would be new in these listings and, we believe, would provide useful adjudicative guidance that is lacking in our current rules.

Section 14.00G has six subsections. The first two (proposed 14.00G1 and 14.00G2) and the last one (proposed 14.00G6) are applicable to all immune system disorders. Proposed 14.00G314.00G5 provide guidance specific to each of the three main types of immune system disorders: Autoimmune disorders (proposed 14.00G3), immune deficiency disorders, excluding HIV infection (proposed 14.00G4), and HIV infection (proposed 14.00G5).

In proposed 14.00G1, General, we incorporate the first and fifth sentences of current 14.00D7. We believe that this guidance has general applicability to all immune system disorders, not just HIV infection. We first explain that we consider both the effectiveness of your treatment on your signs, symptoms, and laboratory findings, and the negative side effects of your treatment on your functioning. We also explain that we will make every reasonable effort to obtain a specific description of the treatment you receive. Then, we list eight factors we consider when we evaluate your treatment. They are mostly based on factors we mention in the current rule, but we propose to expand the list and in some cases to clarify the existing factors in our current rules. For example, instead of referring only to the ``dosage [and] frequency of administration'' of your treatment, we refer to ``the intrusiveness and complexity of your treatment (the dosing schedule, need for injections, etc).'' In proposed 14.00G1e, we also introduce the term ``variability of your response to treatment,'' a concept we address for HIV infection in current 14.00D7 but that we believe is of particular importance in considering the effects of treatment in all individuals with immune system disorders. We explain this concept in more detail in proposed 14.00G2.

Proposed 14.00G1f is new. It describes the interactive and cumulative effects of treatments for immune system disorders and other disorders that people with immune system disorders may also have. We explain that the effects of these treatments taken together may be greater than they would be if we considered them separately, and we provide an example of treatment for HIV infection together with treatment for hepatitis C. Proposed 14.00G1g is also new. It explains that we will also consider the duration of your treatment. Proposed 14.00G1h is a catchall for other relevant factors we have not listed in 14.00G1a14.00G1g.

In proposed 14.00G2, Variability of your response to treatment, we explain what we mean by this factor in terms of both HIV infection and other immune
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system disorders. This proposed rule is based on the language of the second paragraph in current 14.00D7 and the second sentence of the third paragraph of that section. However, we propose to expand that guidance and to apply it to all other immune system disorders in addition to HIV infection. For example, we explain in a general way applicable to all immune system disorders that some individuals may show an initial positive response to drug treatment (or a combination of drugs), but the initial positive response may be followed by a decrease in the effectiveness of the medication.

We provide more specific information about treatment of autoimmune disorders in proposed 14.00G3, How we evaluate the effects of treatment for autoimmune disorders on your ability to function. This proposed rule repeats the rule in the fifth paragraph of current 14.00B that, when we evaluate the effects of your treatment for your autoimmune disorder(s), we will consider the adverse effects that may result in loss of function. We propose to expand this guidance to include more examples of potential chronic adverse effects of steroid treatment and to explain that the side effects of some medications may be acute or longterm. We also propose to add a provision that recognizes that the medications used in the treatment of autoimmune disorders may have effects on mental function, including cognition (memory),

concentration, and mood.

Proposed 14.00G4, How we evaluate the effects of treatment for immune deficiency disorders, excluding HIV infection, on your ability to function, is new. As in proposed 14.00G3, we repeat the principle that we will consider the side effects of your treatment when we evaluate your ability to function. We cite intravenous immunoglobulin and gamma interferon therapy as examples of treatment you may be receiving. We also provide examples of side effects of treatment for immune deficiency disorders, including physical symptoms (such as fatigue and headaches), clinical signs (such as high blood pressure and joint swelling), and limitations in mental function, including cognition, concentration, and mood.

Proposed 14.00G5, How we evaluate the effects of treatment for HIV infection on your ability to function, is in two parts. In proposed 14.00G5a, General, as in proposed 14.00G3 and 14.00G4, we repeat the principle from 14.00D7 that we consider the side effects of antiretroviral treatment and treatment for the manifestation of HIV infection on your ability to function. We propose to expand our guidance to provide examples of the physical and mental side effects of antiretroviral drugs. We also note that the symptoms of HIV infection and the side effects of medications may be indistinguishable, but that we will consider your functional limitations whether they are a result of your symptoms from HIV infection or the side effects of your treatment.

In proposed 14.00G5b, Structured treatment interruptions, we provide new guidance specifically about structured treatment interruptions (STIs, also called drug holidays) in individuals with HIV infection. The proposed guidance clarifies that STIs are part of a prescribed treatment plan and do not show that an individual is failing to follow treatment, or in themselves establish that an individual's impairment is not as severe as alleged.

In proposed 14.00G6, When there is no record of ongoing treatment, we explain how we will evaluate the medical severity and duration of your immune system disorder when you have not received ongoing treatment or have not had an ongoing relationship with any treatment source despite the existence of a severe impairment(s). The provision is based on a standard provision we include in most other body systems listings, for example, 1.00H3 in the musculoskeletal system, the third paragraph of 3.00A in the respiratory system, and the third paragraph of 4.00B3 in the cardiovascular system. We also explain that if you have just begun treatment and we cannot decide whether you are disabled based on the evidence we have, we may need to wait to determine the effect of your treatment. We explain that there is no set period because how long we may need to wait will depend on the facts of your individual case. This is consistent with the guidance we provide in the last sentence of the third paragraph in current 14.00D7, which explains we should decide the impact of treatment based on a sufficient period of treatment.
Proposed 14.00HHow do we consider your symptoms, including your constitutional symptoms or pain?

Proposed 14.00H is new. In it, we explain that we will evaluate the impact your symptoms have on your ability to function when the evidence of your immune system disorder(s) shows that you have a medically determinable impairment that could reasonably be expected to produce your symptoms.
Proposed 14.00IHow do we use the functional criteria in these listings?

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

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

A number of the commenters pointed with approval to the provisions of current listing 14.08N and the text in current 14.00D8 that explains that listing. These individuals thought that the provisions should not be confined to people who have HIV infection but should be extended to people with other kinds of immune system disorders who may be continuously limited by their symptoms and other mani

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