Appendix A
Current HIV Infection Listings (14.08 and 114.08)

CURRENT LISTING OF IMPAIRMENTS

Part A

The following sections in Part A are applicable to individuals age 18 and over and to children under age 18 where criteria are appropriate.

Section

1.00

Musculoskeletal System

2.00

Special Senses and Speech

3.00

Respiratory System

4.00

Cardiovascular System

5.00

Digestive System

6.00

Genitourinary Impairments

7.00

Hematological Disorders

8.00

Skin Disorders

9.00

Endocrine System

10.00

Impairments That Affect Multiple Body Systems

11.00

Neurological

12.00

Mental Disorders

13.00

Malignant Neoplastic Diseases

14.00

Immune System Disorders



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Appendix A Current HIV Infection Listings (14.08 and 114.08) CURRENT LISTING OF IMPAIRMENTS Part A The following sections in Part A are applicable to individuals age 18 and over and to children under age 18 where criteria are appropriate. Section 1.00 Musculoskeletal System 2.00 Special Senses and Speech 3.00 Respiratory System 4.00 Cardiovascular System 5.00 Digestive System 6.00 Genitourinary Impairments 7.00 Hematological Disorders 8.00 Skin Disorders 9.00 Endocrine System 10.00 Impairments That Affect Multiple Body Systems 11.00 Neurological 12.00 Mental Disorders 13.00 Malignant Neoplastic Diseases 14.00 Immune System Disorders 0

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0 HIV AND DISABILITY HIV Infection Listing Introductory Text 14.00A. What disorders do we ealuate under the immune system disorders listings? 1. We ealuate immune system disorders that cause dysfunction in one or more components of your immune system. a. The dysfunction may be due to problems in antibody production, impaired cell mediated immunity, a combined type of antibody/cellular deficiency, impaired phagocytes, or complement deficiency. b. Immune system disorders may result in recurrent and unusual infections, or inflammation and dysfunction of the body’s own tissues. Im- mune system disorders can cause a deficit in a single organ or body system that results in extreme (that is, very serious) loss of function. They can also cause lesser degrees of limitations in two or more organs or body systems, and when associated with symptoms or signs, such as severe fatigue, fever, malaise, diffuse musculoskeletal pain, or involuntary weight loss, can also result in extreme limitation. c. We organize the discussions of immune system disorders in three categories: autoimmune disorders; immune deficiency disorders, excluding human immunodeficiency virus (HIV) infection; and HIV infection. 2. Autoimmune disorders (4.00D). Autoimmune disorders are caused by dysfunctional immune responses directed against the body’s own tissues, resulting in chronic, multisystem impairments that differ in clinical manifes- tations, course, and outcome. They are sometimes referred to as rheumatic diseases, connective tissue disorders, or collagen vascular disorders. Some of the features of autoimmune disorders in adults differ from the features of the same disorders in children. 3. Immune deficiency disorders, excluding HIV infection (4.00E). Im- mune deficiency disorders are characterized by recurrent or unusual in- fections that respond poorly to treatment, and are often associated with complications affecting other parts of the body. Immune deficiency disor- ders are classified as either primary (congenital) or acquired. Individuals with immune deficiency disorders also have an increased risk of malignan- cies and of having autoimmune disorders. 4. Human immunodeficiency irus (HIV) infection (4.00F). HIV infec- tion may be characterized by increased susceptibility to opportunistic infec- tions, cancers, or other conditions, as described in 14.08. B. What information do we need to show that you hae an immune system disorder? Generally, we need your medical history, a report(s) of a

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 APPENDIX A physical examination, a report(s) of laboratory findings, and in some in- stances, appropriate medically acceptable imaging or tissue biopsy reports to show that you have an immune system disorder. Therefore, we will make every reasonable effort to obtain your medical history, medical findings, and results of laboratory tests. We explain the information we need in more detail in the sections below. C. Definitions 1. Appropriate medically acceptable imaging includes, but is not limited to, angiography, X-ray imaging, computerized axial tomography (CAT scan) or magnetic resonance imaging (MRI), with or without contrast mate- rial, myelography, and radionuclear bone scans. “Appropriate” means that the technique used is the proper one to support the evaluation and diagnosis of the impairment. 2. Constitutional symptoms or signs, as used in these listings, means se- vere fatigue, fever, malaise, or involuntary weight loss. Seere fatigue means a frequent sense of exhaustion that results in significantly reduced physi- cal activity or mental function. Malaise means frequent feelings of illness, bodily discomfort, or lack of well-being that result in significantly reduced physical activity or mental function. 3. Disseminated means that a condition is spread over a considerable area. The type and extent of the spread will depend on your specific disease. 4. Dysfunction means that one or more of the body regulatory mecha- nisms are impaired, causing either an excess or deficiency of immunocom- petent cells or their products. 5. Extra-articular means “other than the joints”; for example, an organ(s) such as the heart, lungs, kidneys, or skin. 6. Inability to ambulate effectiely has the same meaning as in 1.00B2b. 7. Inability to perform fine and gross moements effectiely has the same meaning as in 1.00B2c. 8. Major peripheral joints has the same meaning as in 1.00F. 9. Persistent means that a sign(s) or symptom(s) has continued over time. The precise meaning will depend on the specific immune system disorder, the usual course of the disorder, and the other circumstances of your clinical course.

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 HIV AND DISABILITY 10. Recurrent means that a condition that previously responded adequately to an appropriate course of treatment returns after a period of remission or regression. The precise meaning, such as the extent of response or remission and the time periods involved, will depend on the specific disease or condi- tion you have, the body system affected, the usual course of the disorder and its treatment, and the other facts of your particular case. 11. Resistant to treatment means that a condition did not respond ad- equately to an appropriate course of treatment. Whether a response is adequate or a course of treatment is appropriate will depend on the specific disease or condition you have, the body system affected, the usual course of the disorder and its treatment, and the other facts of your particular case. 12. Seere means medical severity as used by the medical community. The term does not have the same meaning as it does when we use it in connec- tion with a finding at the second step of the sequential evaluation processes in §§404.1520, 416.920, and 416.924. D. Refers to autoimmune disorders excluding HIV. E. Refers to immune deficiency disorders excluding HIV. F. How do we document and ealuate human immunodeficiency irus (HIV) infection? Any individual with HIV infection, including one with a diagnosis of acquired immune deficiency syndrome (AIDS), may be found disabled under 14.08 if his or her impairment meets the criteria in that list- ing or is medically equivalent to the criteria in that listing. 1. Documentation of HIV infection. The medical evidence must include documentation of HIV infection. Documentation may be by laboratory evidence or by other generally acceptable methods consistent with the pre- vailing state of medical knowledge and clinical practice. When you have had laboratory testing for HIV infection, we will make every reasonable effort to obtain reports of the results of that testing. However, we will not purchase laboratory testing to establish whether you have HIV infection. a. Definitie documentation of HIV infection. A definitive diagnosis of HIV infection is documented by one or more of the following laboratory tests: (i) HIV antibody tests. HIV antibodies are usually first detected by an ELISA screening test performed on serum. Because the ELISA can yield false-positive results, confirmation is required using a more definitive test, such as a Western blot or an immunofluorescence assay. (ii) Positive “viral load” (VL) tests. These tests are normally used to quantitate the amount of the virus present, but also document HIV infec-

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 APPENDIX A tion. Such tests include the quantitative plasma HIV RNA, quantitative plasma HIV branched DNA, and reverse transcriptase-polymerase chain reaction (RT-PCR). (iii) HIV DNA detection by polymerase chain reaction (PCR). (iv) A specimen that contains HIV antigen (for example, serum speci- men, lymphocyte culture, or cerebrospinal fluid). (v) A positive viral culture for HIV from peripheral blood mono- nuclear cells (PBMCs). (vi) Other tests that are highly specific for detection of HIV and that are consistent with the prevailing state of medical knowledge. b. Other acceptable documentation of HIV infection. We may also document HIV infection without the definitive laboratory evidence de- scribed in 14.00F1a, provided that such documentation is consistent with the prevailing state of medical knowledge and clinical practice and is con- sistent with the other evidence in your case record. If no definitive labora- tory evidence is available, we may document HIV infection by the medical history, clinical and laboratory findings, and diagnosis(es) indicated in the medical evidence. For example, we will accept a diagnosis of HIV infection without definitive laboratory evidence of the HIV infection if you have an opportunistic disease that is predictive of a defect in cell-mediated immunity (for example, toxoplasmosis of the brain, Pneumocystis pneumonia (PCP)), and there is no other known cause of diminished resistance to that disease (for example, long-term steroid treatment, lymphoma). In such cases, we will make every reasonable effort to obtain full details of the history, medi- cal findings, and results of testing. 2. CD4 tests. Individuals who have HIV infection or other disorders of the immune system may have tests showing a reduction of either the absolute count or the percentage of their T-helper lymphocytes (CD4 cells). The ex- tent of immune suppression correlates with the level or rate of decline of the CD4 count. Generally, when the CD4 count is below 200/mm3 (or below 14 percent of the total lymphocyte count), the susceptibility to opportunistic infection is greatly increased. Although a reduced CD4 count alone does not establish a definitive diagnosis of HIV infection, a CD4 count below 200 does offer supportive evidence when there are clinical findings, but not a de- finitive diagnosis of an opportunistic infection(s). However, a reduced CD4 count alone does not document the severity or functional consequences of HIV infection. 3. Documentation of the manifestations of HIV infection. The medical evidence must also include documentation of the manifestations of HIV infection. Documentation may be by laboratory evidence or other generally

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4 HIV AND DISABILITY acceptable methods consistent with the prevailing state of medical knowl- edge and clinical practice. a. Definitie documentation of the manifestations of HIV infection. The definitive method of diagnosing opportunistic diseases or conditions that are manifestations of HIV infection is by culture, serologic test, or microscopic examination of biopsied tissue or other material (for example, bronchial washings). We will make every reasonable effort to obtain spe- cific laboratory evidence of an opportunistic disease or other condition whenever this information is available. If a histologic or other test has been performed, the evidence should include a copy of the appropriate report. If we cannot obtain the report, the summary of hospitalization or a report from the treating source should include details of the findings and results of the diagnostic studies (including appropriate medically acceptable imag- ing studies) or microscopic examination of the appropriate tissues or body fluids. b. Other acceptable documentation of the manifestations of HIV infection. We may also document manifestations of HIV infection without the definitive laboratory evidence described in 14.00F3a, provided that such documentation is consistent with the prevailing state of medical knowledge and clinical practice and is consistent with the other evidence in your case record. For example, many conditions are now commonly diagnosed based on some or all of the following: medical history, clinical manifestations, laboratory findings (including appropriate medically acceptable imaging), and treatment responses. In such cases, we will make every reasonable effort to obtain full details of the history, medical findings, and results of testing. The following are examples of how we may document manifesta- tions of HIV infection with other appropriate evidence. (i) Although a definitive diagnosis of PCP requires identifying the organism in bronchial washings, induced sputum, or lung biopsy, these tests are frequently bypassed if PCP can be diagnosed presumptively. Sup- portive evidence may include fever, dyspnea, hypoxia, CD4 count below 200, and no evidence of bacterial pneumonia. Also supportive are bilateral lung interstitial infiltrates on X-ray, a typical pattern on CAT scan, or a gallium scan positive for pulmonary uptake. Response to anti-PCP therapy usually requires 5–7 days, and such a response can be supportive of the diagnosis. (ii) Documentation of Cytomegaloirus (CMV) disease (14.08D) may present special problems because definitive diagnosis (except for chorio- retinitis, which may be diagnosed by an ophthalmologist or optometrist on funduscopic examination) requires identification of viral inclusion bodies or a positive culture from the affected organ and the absence of any other infectious agent likely to be causing the disease. A positive serology test does not establish a definitive diagnosis of CMV disease, but does offer

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 APPENDIX A supportive evidence of a presumptive diagnosis of CMV disease. Other clinical findings that support a presumptive diagnosis of CMV may include: fever, urinary culture positive for CMV, and CD4 count below 200. A clear response to anti-CMV therapy also supports a diagnosis. (iii) A definitive diagnosis of toxoplasmosis of the brain is based on brain biopsy, but this procedure carries significant risk and is not com- monly performed. This condition is usually diagnosed presumptively based on symptoms or signs of fever, headache, focal neurologic deficits, seizures, typical lesions on brain imaging, and a positive serology test. (iv) Candidiasis of the esophagus (also known as Candida esophagi- tis) may be presumptively diagnosed based on symptoms of retrosternal pain on swallowing (odynophagia) and either oropharyngeal thrush (white patches or plaques) diagnosed on physical examination or by microscopic documentation of Candida fungal elements from a noncultured specimen scraped from the oral mucosa. Treatment with oral (systemic) antifungal agents usually produces improvement after 5 or more days of therapy, and such a response can be supportive of the diagnosis. 4. HIV infection manifestations specific to women. a. General. Most women with severe immunosuppression second- ary to HIV infection exhibit the typical opportunistic infections and other conditions, such as PCP, Candida esophagitis, wasting syndrome, crypto- coccosis, and toxoplasmosis. However, HIV infection may have different manifestations in women than in men. Adjudicators must carefully scruti- nize the medical evidence and be alert to the variety of medical conditions specific to, or common in, women with HIV infection that may affect their ability to function in the workplace. b. Additional considerations for ealuating HIV infection in women. Many of these manifestations (for example, vulvovaginal candidiasis, pelvic inflammatory disease) occur in women with or without HIV infection, but can be more severe or resistant to treatment or occur more frequently in a woman whose immune system is suppressed. Therefore, when evaluating the claim of a woman with HIV infection, it is important to consider gy- necologic and other problems specific to women, including any associated symptoms (for example, pelvic pain), in assessing the severity of the impair- ment and resulting functional limitations. We may evaluate manifestations of HIV infection in women under the specific criteria (for example, cervical cancer under 14.08E), under an applicable general category (for example, pelvic inflammatory disease under 14.08A4), or, in appropriate cases, under 14.08K. 5. Inoluntary weight loss. For purposes of 14.08H, an involuntary weight loss of at least 10 percent of baseline is always considered “significant.”

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 HIV AND DISABILITY Loss of less than 10 percent may or may not be significant, depending on the individual’s baseline weight and body habitus. For example, a 7-pound weight loss in a 100-pound woman who is 63 inches tall might be consid- ered significant; but a 14-pound weight loss in a 200-pound woman who is the same height might not be significant. HIV infection that affects the digestive system and results in malnutrition can also be evaluated under 5.08. G. How do we consider the effects of treatment in ealuating your autoim- mune disorder, immune deficiency disorder, or HIV infection? 1. General. If your impairment does not otherwise meet the requirements of a listing, we will consider your medical treatment in terms of its effec- tiveness in improving the signs, symptoms, and laboratory abnormalities of your specific immune system disorder or its manifestations, and in terms of any side effects that limit your functioning. We will make every reasonable effort to obtain a specific description of the treatment you receive (including surgery) for your immune system disorder. We consider: a. The effects of medications you take. b. Adverse side effects (acute and chronic). c. The intrusiveness and complexity of your treatment (for example, the dosing schedule, need for injections). d. The effect of treatment on your mental functioning (for example, cognitive changes, mood disturbance). e. Variability of your response to treatment (see 14.00G2). f. The interactive and cumulative effects of your treatments. For ex- ample, many individuals with immune system disorders receive treatment both for their immune system disorders and for the manifestations of the disorders or co-occurring impairments, such as treatment for HIV infection and hepatitis C. The interactive and cumulative effects of these treatments may be greater than the effects of each treatment considered separately. g. The duration of your treatment. h. Any other aspects of treatment that may interfere with your ability to function. 2. Variability of your response to treatment. Your response to treatment and the adverse or beneficial consequences of your treatment may vary widely. The effects of your treatment may be temporary or long term. For example, some individuals may show an initial positive response to a drug or combination of drugs followed by a decrease in effectiveness. When we evaluate your response to treatment and how your treatment may af- fect you, we consider such factors as disease activity before treatment, requirements for changes in therapeutic regimens, the time required for therapeutic effectiveness of a particular drug or drugs, the limited number

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 APPENDIX A of drug combinations that may be available for your impairment(s), and the time-limited efficacy of some drugs. For example, an individual with HIV infection or another immune deficiency disorder who develops pneumonia or tuberculosis may not respond to the same antibiotic regimen used in treating individuals without HIV infection or another immune deficiency disorder, or may not respond to an antibiotic that he or she responded to before. Therefore, we must consider the effects of your treatment on an individual basis, including the effects of your treatment on your ability to function. 3. How we ealuate the effects of treatment for autoimmune disorders on your ability to function. Some medications may have acute or long-term side effects. When we consider the effects of corticosteroids or other treat- ments for autoimmune disorders on your ability to function, we consider the factors in 14.00G1 and 14.00G2. Long-term corticosteroid treatment can cause ischemic necrosis of bone, posterior subcapsular cataract, weight gain, glucose intolerance, increased susceptibility to infection, and osteo- porosis that may result in a loss of function. In addition, medications used in the treatment of autoimmune disorders may also have effects on mental functioning, including cognition (for example, memory), concentration, and mood. 4. Refers to effects of treatment excluding HIV. 5. How we ealuate the effects of treatment for HIV infection on your ability to function. a. General. When we consider the effects of antiretroviral drugs (in- cluding the effects of highly active antiretroviral therapy (HAART)) and the effects of treatments for the manifestations of HIV infection on your ability to function, we consider the factors in 14.00G1 and 14.00G2. Side effects of antiretroviral drugs include, but are not limited to: bone marrow suppression, pancreatitis, gastrointestinal intolerance (nausea, vomiting, diarrhea), neuropathy, rash, hepatotoxicity, lipodystrophy (fat redistribu- tion, such as “buffalo hump”), glucose intolerance, and lactic acidosis. In addition, medications used in the treatment of HIV infection may also have effects on mental functioning, including cognition (for example, memory), concentration, and mood, and may result in malaise, severe fatigue, joint and muscle pain, and insomnia. The symptoms of HIV infection and the side effects of medication may be indistinguishable from each other. We will consider all of your functional limitations, whether they result from your symptoms or signs of HIV infection or the side effects of your treatment. b. Structured treatment interruptions. A structured treatment inter- ruption (STI, also called a “drug holiday”) is a treatment practice during

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 HIV AND DISABILITY which your treating source advises you to stop taking your medications temporarily. An STI in itself does not imply that your medical condition has improved, nor does it imply that you are noncompliant with your treatment because you are following your treating source’s advice. Therefore, if you have stopped taking medication because your treating source prescribed or recommended an STI, we will not find that you are failing to follow treat- ment or draw inferences about the severity of your impairment on this fact alone. We will consider why your treating source has prescribed or recom- mended an STI and all the other information in your case record when we determine the severity of your impairment. 6. When there is no record of ongoing treatment. If you have not re- ceived ongoing treatment or have not had an ongoing relationship with the medical community despite the existence of a severe impairment(s), we will evaluate the medical severity and duration of your immune system disorder on the basis of the current objective medical evidence and other evidence in your case record, taking into consideration your medical history, symptoms, clinical and laboratory findings, and medical source opinions. If you have just begun treatment and we cannot determine whether you are disabled based on the evidence we have, we may need to wait to determine the effect of the treatment on your ability to function. The amount of time we need to wait will depend on the facts of your case. If you have not received treat- ment, you may not be able to show an impairment that meets the criteria of one of the immune system disorders listings, but your immune system disor- der may medically equal a listing or be disabling based on a consideration of your residual functional capacity, age, education, and work experience. H. How do we consider your symptoms, including your pain, seere fatigue, and malaise? Your symptoms, including pain, severe fatigue, and malaise, may be important factors in our determination whether your immune system disorder(s) meets or medically equals a listing or in our determination whether you are otherwise able to work. In order for us to consider your symptoms, you must have medical signs or laboratory find- ings showing the existence of a medically determinable impairment(s) that could reasonably be expected to produce the symptoms. If you have such an impairment(s), we will evaluate the intensity, persistence, and functional effects of your symptoms using the rules throughout 14.00 and in our other regulations. See §§404.1528, 404.1529, 416.928, and 416.929. Addition- ally, when we assess the credibility of your complaints about your symp- toms and their functional effects, we will not draw any inferences from the fact that you do not receive treatment or that you are not following treat- ment 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.

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 APPENDIX A I. How do we use the functional criteria in these listings? 1. The following listings in this body system include standards for evalu- ating the functional limitations resulting from immune system disorders: 14.02B, for systemic lupus erythematosus; 14.03B, for systemic vasculitis; 14.04D, for systemic sclerosis (scleroderma); 14.05E, for polymyositis and dermatomyositis; 14.06B, for undifferentiated and mixed connective tissue disease; 14.07C, for immune deficiency disorders, excluding HIV infection; 14.08K, for HIV infection; 14.09D, for inflammatory arthritis; and 14.10B, for Sjögren’s syndrome. 2. When we use one of the listings cited in 14.00I1, we will consider all relevant information in your case record to determine the full impact of your immune system disorder on your ability to function on a sustained basis. Important factors we will consider when we evaluate your function- ing under these listings include, but are not limited to: your symptoms, the frequency and duration of manifestations of your immune system disorder, periods of exacerbation and remission, and the functional impact of your treatment, including the side effects of your medication. 3. As used in these listings, “repeated” means that the manifestations oc- cur on an average of three times a year, or once every 4 months, each lasting 2 weeks or more; or the manifestations do not last for 2 weeks, but occur substantially more frequently than three times in a year or once every 4 months; or they occur less frequently than an average of three times a year or once every 4 months, but last substantially longer than 2 weeks. Your impairment will satisfy this criterion regardless of whether you have the same kind of manifestation repeatedly, all different manifestations, or any other combination of manifestations; for example, two of the same kind of manifestation and a different one. You must have the required number of manifestations with the frequency and duration required in this section. Also, the manifestations must occur within the period covered by your claim. 4. To satisfy the functional criterion in a listing, your immune system dis- order must result in a “marked” level of limitation in one of three general areas of functioning: Activities of daily living, social functioning, or difficul- ties in completing tasks due to deficiencies in concentration, persistence, or pace. Functional limitation may result from the impact of the disease process itself on your mental functioning, physical functioning, or both your mental and physical functioning. This could result from persistent or intermittent symptoms, such as depression, severe fatigue, or pain, resulting in a limita- tion of your ability to do a task, to concentrate, to persevere at a task, or to perform the task at an acceptable rate of speed. You may also have limita- tions because of your treatment and its side effects (see 14.00G).

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0 HIV AND DISABILITY microscopic examination of biopsied tissue or other material (for example, bronchial washings). We will make every reasonable effort to obtain spe- cific laboratory evidence of an opportunistic disease or other condition whenever this information is available. If a histologic or other test has been performed, the evidence should include a copy of the appropriate report. If we cannot obtain the report, the summary of hospitalization or a report from the treating source should include details of the findings and results of the diagnostic studies (including appropriate medically acceptable imag- ing studies) or microscopic examination of the appropriate tissues or body fluids. b. Other acceptable documentation of the manifestations of HIV in- fection. We may also document manifestations of HIV infection without the definitive laboratory evidence described in 114.00F3a, provided that such documentation is consistent with the prevailing state of medical knowledge and clinical practice and is consistent with the other evidence in your case record. For example, many conditions are now commonly diagnosed based on some or all of the following: Medical history, clinical manifestations, laboratory findings (including appropriate medically acceptable imaging), and treatment responses. In such cases, we will make every reasonable effort to obtain full details of the history, medical findings, and results of testing. The following are examples of how we may document manifesta- tions of HIV infection with other appropriate evidence. (i) Although a definitive diagnosis of PCP requires identifying the or- ganism in bronchial washings, induced sputum, or lung biopsy, these tests are frequently bypassed if PCP can be diagnosed presumptively. Supportive evidence may include: Fever, dyspnea, hypoxia, CD4 count below 200 in children 6 years of age or older, and no evidence of bacterial pneumonia. Also supportive are bilateral lung interstitial infiltrates on X-ray, a typical pattern on CAT scan, or a gallium scan positive for pulmonary uptake. Re- sponse to anti-PCP therapy usually requires 5-7 days, and such a response can be supportive of the diagnosis. (ii) Documentation of Cytomegaloirus (CMV) disease (114.08D) may present special problems because definitive diagnosis (except for chorio- retinitis, which may be diagnosed by an ophthalmologist or optometrist on funduscopic examination) requires identification of viral inclusion bodies or a positive culture from the affected organ and the absence of any other infectious agent likely to be causing the disease. A positive serology test does not establish a definitive diagnosis of CMV disease, but does offer sup- portive evidence of a presumptive diagnosis of CMV disease. Other clinical findings that support a presumptive diagnosis of CMV may include: Fever, urinary culture positive for CMV, and CD4 count below 200 in children 6 years of age or older. A clear response to anti-CMV therapy also supports a diagnosis.

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 APPENDIX A (iii) A definitive diagnosis of toxoplasmosis of the brain is based on brain biopsy, but this procedure carries significant risk and is not com- monly performed. This condition is usually diagnosed presumptively based on symptoms or signs of fever, headache, focal neurologic deficits, seizures, typical lesions on brain imaging, and a positive serology test. (iv) Candidiasis of the esophagus (also known as Candida esophagi- tis) may be presumptively diagnosed based on symptoms of retrosternal pain on swallowing (odynophagia) and either oropharyngeal thrush (white patches or plaques) diagnosed on physical examination or by microscopic documentation of Candida fungal elements from a noncultured specimen scraped from the oral mucosa. Treatment with oral (systemic) antifungal agents usually produces improvement after 5 or more days of therapy, and such a response can be supportive of the diagnosis. 4. HIV infection manifestations specific to children. a. General. The clinical manifestation and course of disease in chil- dren who become infected with HIV perinatally or in the first 12 years of life may differ from that in adolescents (age 12 to attainment of age 18) and adults. Newborn and younger infants (birth to attainment of age 1) and older infants and toddlers (age 1 to attainment of age 3) may present with failure to thrive or PCP; preschool children (age 3 to attainment of age 6) and primary school children (age 6 to attainment of age 12) may present with recurrent infections, neurological problems, or developmental abnor- malities. Adolescents may also exhibit neurological abnormalities, such as HIV encephalopathy, or have growth problems. HIV infection that affects the digestive system and results in malnutrition also may be evaluated under 105.08. b. Neurologic abnormalities. The methods of identifying and evalu- ating neurologic abnormalities may vary depending on a child’s age. For example, in an infant, impaired brain growth can be documented by a decrease in the growth rate of the head. In an older child, impaired brain growth may be documented by brain atrophy on a CAT scan or MRI. Neu- rologic abnormalities in infants and young children may present as serious developmental delays or in the loss of previously acquired developmental milestones. In school-age children and adolescents, this type of neurologic abnormality generally presents as the loss of previously acquired intellec- tual abilities. This may be evidenced in a child by a decrease in intelligence quotient (IQ) scores, by forgetting information previously learned, by in- ability to learn new information, or by a sudden onset of a new learning disability. c. Bacterial infections. Children with HIV infection may contract any of a broad range of bacterial infections. Certain major infections caused by pyogenic bacteria (for example, some pneumonias) can be severely limit-

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 HIV AND DISABILITY ing, especially in pre-adolescent children. We evaluate these major bacterial infections under 114.08A4. Although 114.08A4 applies only to children under 13 years of age, children age 13 and older may have an impairment that medically equals this listing if the circumstances of the case warrant; for example, if there is delayed puberty. We will evaluate pelvic inflamma- tory disease in older girls under 114.08A5. G. How do we consider the effects of treatment in ealuating your autoim- mune disorder, immune deficiency disorder, or HIV infection? 1. General. If your impairment does not otherwise meet the requirements of a listing, we will consider your medical treatment in terms of its effec- tiveness in improving the signs, symptoms, and laboratory abnormalities of your specific immune system disorder or its manifestations, and in terms of any side effects that limit your functioning. We will make every reasonable effort to obtain a specific description of the treatment you receive (including surgery) for your immune system disorder. We consider: a. The effects of medications you take. b. Adverse side effects (acute and chronic). c. The intrusiveness and complexity of your treatment (for example, the dosing schedule, need for injections). d. The effect of treatment on your mental functioning (for example, cognitive changes, mood disturbance). e. Variability of your response to treatment (see 114.00G2). f. The interactive and cumulative effects of your treatments. For example, many children with immune system disorders receive treatment both for their immune system disorders and for the manifestations of the disorders or co-occurring impairments, such as treatment for HIV infection and hepatitis C. The interactive and cumulative effects of these treatments may be greater than the effects of each treatment considered separately. g. The duration of your treatment. h. Any other aspects of treatment that may interfere with your ability to function. 2. Variability of your response to treatment. Your response to treatment and the adverse or beneficial consequences of your treatment may vary widely. The effects of your treatment may be temporary or long term. For example, some children may show an initial positive response to a drug or combination of drugs followed by a decrease in effectiveness. When we evaluate your response to treatment and how your treatment may af- fect you, we consider such factors as disease activity before treatment, requirements for changes in therapeutic regimens, the time required for therapeutic effectiveness of a particular drug or drugs, the limited number of drug combinations that may be available for your impairment(s), and

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 APPENDIX A the time-limited efficacy of some drugs. For example, a child with HIV infection or another immune deficiency disorder who develops otitis media may not respond to the same antibiotic regimen used in treating children without HIV infection or another immune deficiency disorder, or may not respond to an antibiotic that he or she responded to before. Therefore, we must consider the effects of your treatment on an individual basis, including the effects of your treatment on your ability to function. 3. How we ealuate the effects of treatment for autoimmune disorders on your ability to function. Some medications may have acute or long-term side effects. When we consider the effects of corticosteroids or other treat- ments for autoimmune disorders on your ability to function, we consider the factors in 114.00G1 and 114.00G2. Long-term corticosteroid treatment can cause ischemic necrosis of bone, posterior subcapsular cataract, im- paired growth, weight gain, glucose intolerance, increased susceptibility to infection, and osteopenia that may result in a loss of function. In addition, medications used in the treatment of autoimmune disorders may also have effects on mental functioning, including cognition (for example, memory), concentration, and mood. 4. How we ealuate the effects of treatment for immune deficiency dis- orders, excluding HIV infection, on your ability to function. When we consider the effects of your treatment for your immune deficiency disor- der on your ability to function, we consider the factors in 114.00G1 and 114.00G2. A frequent need for treatment such as intravenous immuno- globulin and gamma interferon therapy can be intrusive and interfere with your ability to function. We will also consider whether you have chronic side effects from these or other medications, including severe fatigue, fever, headaches, high blood pressure, joint swelling, muscle aches, nausea, short- ness of breath, or limitations in mental function including cognition (for example, memory) concentration, and mood. 5. How we ealuate the effects of treatment for HIV infection on your ability to function. a. General. When we consider the effects of antiretroviral drugs (in- cluding the effects of highly active antiretroviral therapy (HAART)) and the effects of treatments for the manifestations of HIV infection on your ability to function, we consider the factors in 114.00G1 and 114.00G2. Side ef- fects of antiretroviral drugs include, but are not limited to: Bone marrow suppression, pancreatitis, gastrointestinal intolerance (nausea, vomiting, diarrhea), neuropathy, rash, hepatotoxicity, lipodystrophy (fat redistribu- tion, such as “buffalo hump”), glucose intolerance, and lactic acidosis. In addition, medications used in the treatment of HIV infection may also have

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4 HIV AND DISABILITY effects on mental functioning, including cognition (for example, memory), concentration, and mood, and may result in malaise, severe fatigue, joint and muscle pain, and insomnia. The symptoms of HIV infection and the side effects of medication may be indistinguishable from each other. We will consider all of your functional limitations, whether they result from your symptoms or signs of HIV infection or the side effects of your treatment. b. Structured treatment interruptions. A structured treatment inter- ruption (STI, also called a “drug holiday”) is a treatment practice during which your treating source advises you to stop taking your medications temporarily. An STI in itself does not imply that your medical condition has improved; nor does it imply that you are noncompliant with your treatment because you are following your treating source’s advice. Therefore, if you have stopped taking medication because your treating source prescribed or recommended an STI, we will not find that you are failing to follow treat- ment or draw inferences about the severity of your impairment on this fact alone. We will consider why your treating source has prescribed or recom- mended an STI and all the other information in your case record when we determine the severity of your impairment. 6. When there is no record of ongoing treatment. If you have not re- ceived ongoing treatment or have not had an ongoing relationship with the medical community despite the existence of a severe impairment(s), we will evaluate the medical severity and duration of your immune system disorder on the basis of the current objective medical evidence and other evidence in your case record, taking into consideration your medical history, symp- toms, clinical and laboratory findings, and medical source opinions. If you have just begun treatment and we cannot determine whether you are disabled based on the evidence we have, we may need to wait to determine the effect of the treatment on your ability to develop and function in an age-appropriate manner. The amount of time we need to wait will depend on the facts of your case. If you have not received treatment, you may not be able to show an impairment that meets the criteria of one of the immune system disorders listings, but your immune system disorder may medically equal a listing or functionally equal the listings. H. How do we consider your symptoms, including your pain, seere fatigue, and malaise? Your symptoms, including pain, severe fatigue, and malaise, may be important factors in our determination whether your immune system disorder(s) meets or medically equals a listing or in our determination whether you otherwise have marked and severe functional limitations. In order for us to consider your symptoms, you must have medical signs or laboratory findings showing the existence of a medically determinable impairment(s) that could reasonably be expected to produce

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 APPENDIX A the symptoms. If you have such an impairment(s), we will evaluate the intensity, persistence, and functional effects of your symptoms using the rules throughout 114.00 and in our other regulations. See §§416.928, and 416.929. Additionally, when we assess the credibility of your complaints about your symptoms and their functional effects, we will not draw any inferences from the fact that you do not receive treatment or that you are not following treatment without considering all of the relevant evidence in your case record, including any explanations you provide that may explain why you are not receiving or following treatment. I. How do we use the functional criteria in these listings? 1. The following listings in this body system include standards for evalu- ating the functional limitations resulting from immune system disorders: 114.02B, for systemic lupus erythematosus; 114.03B, for systemic vasculi- tis; 114.04D, for systemic sclerosis (scleroderma); 114.05E, for polymyositis and dermatomyositis; 114.06B, for undifferentiated and mixed connective tissue disease; 114.07C, for immune deficiency disorders, excluding HIV infection; 114.08L, for HIV infection; 114.09D, for inflammatory arthritis; and 114.10B, for Sjögren’s syndrome. 2. When we use one of the listings cited in 114.00I1, we will consider all relevant information in your case record to determine the full impact of your immune system disorder on your ability to function. Important factors we will consider when we evaluate your functioning under these listings include, but are not limited to: Your symptoms, the frequency and duration of manifestations of your immune system disorder, periods of exacerbation and remission, and the functional impact of your treatment, including the side effects of your medication. 3. To satisfy the functional criterion in a listing, your immune system dis- order must result in an “extreme” limitation in one domain of functioning or a “marked” limitation in two domains of functioning depending on your age. (See 112.00C for additional discussion of these areas of functioning and §§416.924a and 416.926a for additional guidance on the evaluation of functioning in children.) Functional limitation may result from the impact of the disease process itself on your mental functioning, physical function- ing, or both your mental and physical functioning. This could result from persistent or intermittent symptoms, such as depression, severe fatigue, or pain, resulting in a limitation of your ability to do a task, to concentrate, to persevere at a task, or to perform the task at an acceptable rate of speed. You may also have limitations because of your treatment and its side effects (see 114.00G).

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 HIV AND DISABILITY J. How do we ealuate your immune system disorder when it does not meet one of these listings? 1. These listings are only examples of immune system disorders that we consider severe enough to result in marked and severe functional limita- tions. If your impairment(s) does not meet the criteria of any of these listings, we must also consider whether you have an impairment(s) that satisfies the criteria of a listing in another body system. 2. Individuals with immune system disorders, including HIV infection, may manifest signs or symptoms of a mental impairment or of another physical impairment. We may evaluate these impairments under any af- fected body system. For example, we will evaluate: a. Growth impairment under 100.00. b. Musculoskeletal involvement, such as surgical reconstruction of a joint, under 101.00. c. Ocular involvement, such as dry eye, under 102.00. d. Respiratory impairments, such as pleuritis, under 103.00. e. Cardiovascular impairments, such as cardiomyopathy, under 104.00. f. Digestive impairments, such as hepatitis (including hepatitis C) or weight loss as a result of HIV infection that affects the digestive system, under 105.00. g. Genitourinary impairments, such as nephropathy, under 106.00. h. Hematologic abnormalities, such as anemia, granulocytopenia, and thrombocytopenia, under 107.00. i. Skin impairments, such as persistent fungal and other infectious skin eruptions, and photosensitivity, under 108.00. j. Neurologic impairments, such as neuropathy or seizures, under 111.00. k. Mental disorders, such as depression, anxiety, or cognitive deficits, under 112.00. l. Allergic disorders, such as asthma or atopic dermatitis, under 103.00 or 108.00 or under the criteria in another affected body system. m. Syphilis or neurosyphilis under the criteria for the affected body system, for example, 102.00 Special senses and speech, 104.00 Cardiovas- cular system, or 111.00 Neurological. 3. If you have a severe medically determinable impairment(s) that does not meet a listing, we will determine whether your impairment(s) medi- cally equals a listing. (See §416.926.) If it does not, we will also consider whether you have an impairment(s) that functionally equals the listings. (See §416.926a.) We use the rules in §416.994a when we decide whether you continue to be disabled.

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 APPENDIX A HIV Infection Listing 114.08  Human immunodeficiency virus (HIV) infection. With documenta- tion as described in 114.00F and one of the following: A. Bacterial infections: 1. Mycobacterial infection (for example, caused by M. aium-intracellulare, M. kansasii, or M. tuberculosis) at a site other than the lungs, skin, or cervi- cal or hilar lymph nodes, or pulmonary tuberculosis resistant to treatment; or 2. Nocardiosis; or 3. Salmonella bacteremia, recurrent non-typhoid; or 4. In a child less than 13 years of age, multiple or recurrent pyogenic bacterial infections (sepsis, pneumonia, meningitis, bone or joint infection, or abscess of an internal organ or body cavity, but not otitis media or su- perficial skin or mucosal abscesses) occurring two or more times in 2 years (for children age 13 and older, see 114.00F4c); or 5. Multiple or recurrent bacterial infections, including pelvic inflamma- tory disease, requiring hospitalization or intravenous antibiotic treatment three or more times in 12-month period. OR B. Fungal infections: 1. Aspergillosis; or 2. Candidiasis involving the esophagus, trachea, bronchi, or lungs, or at a site other than the skin, urinary tract, intestinal tract, or oral or vulvo- vaginal mucous membranes; or 3. Coccidioidomycosis, at a site other than the lungs or lymph nodes; or 4. Cryptococcosis, at a site other than the lungs (for example, cryptococ- cal meningitis); or 5. Histoplasmosis, at a site other than the lungs or lymph nodes; or 6. Mucormycosis; or 7. Pneumocystis pneumonia or extrapulmonary Pneumocystis infection. OR C. Protozoan or helminthic infections: 1. Cryptosporidiosis, isosporiasis, or microsporidiosis, with diarrhea last- ing for 1 month or longer; or 2. Strongyloidiasis, extra-intestinal; or 3. Toxoplasmosis of an organ other then the liver, spleen, or lymph nodes. OR D. Viral infections: 1. Cytomegaloirus disease (documented as described in 114.00F3b(ii)) at a site other than the liver, spleen, or lymph nodes; or

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 HIV AND DISABILITY 2. Herpes simplex virus causing: a. Mucocutaneous infection (for example, oral, genital, perianal) last- ing for 1 month or longer; or b. Infection at a site other than the skin or mucous membranes (for example, bronchitis, pneumonitis, esophagitis, or encephalitis); or c. Disseminated infection; or 3. Herpes zoster: a. Disseminated; or b. With multidermatomal eruptions that are resistant to treatment; or 4. Progressive multifocal leukoencephalopathy. OR E. Malignant neoplasms: 1. Carcinoma of the cervix, invasive, FIGO stage II and beyond; or 2. Kaposi’s sarcoma with: a. Extensive oral lesions; or b. Involvement of the gastrointestinal tract, lungs, or other visceral organs; or 3. Lymphoma (for example, primary lymphoma of the brain, Burkitt’s lymphoma, immunoblastic sarcoma, other non-Hodgkin’s lymphoma, Hodgkin’s disease); or 4. Squamous cell carcinoma of the anal canal or anal margin. OR F. Conditions of the skin or mucous membranes (other than described in B2, D2, or D3, above), with extensive fungating or ulcerating lesions not responding to treatment (for example, dermatological conditions such as eczema or psoriasis, vulvovaginal or other mucosal Candida, condyloma caused by human Papillomairus, genital ulcerative disease). OR G. Neurological manifestations of HIV infection (for example, HIV en- cephalopathy, peripheral neuropathy) resulting in one of the following: 1. Loss of previously acquired, or marked delay in achieving, developmen- tal milestones or intellectual ability (including the sudden onset of a new learning disability); or 2. Impaired brain growth (acquired microcephaly or brain atrophy—see 114.00F4b); or 3. Progressive motor dysfunction affecting gait and station or fine and gross motor skills. OR H. Growth disturbance, with: 1. An involuntary weight loss (or failure to gain weight at an appropriate rate for age) resulting in a fall of 15 percentiles from established growth curve (on standard growth charts) that persists for 2 months or longer, or

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 APPENDIX A 2. An involuntary weight loss (or failure to gain weight at an appropriate rate for age) resulting in a fall to below the third percentile from an estab- lished growth curve (on standard growth charts) that persists for 2 months or longer; or 3. Involuntary weight loss of 10 percent or more of baseline (computed based on pounds, kilograms, or body mass index (BMI)) that persists for 2 months or longer. OR I. Diarrhea, lasting for 1 month or longer, resistant to treatment and re- quiring intravenous hydration, intravenous alimentation, or tube feeding. OR J. Lymphoid interstitial pneumonia/pulmonary lymphoid hyperplasia (LIP/PLH complex), with respiratory symptoms that significantly interfere with age-appropriate activities, and that cannot be controlled by prescribed treatment. OR K. One or more of the following infections (other than described in A-J, above). The infection(s) must either be resistant to treatment or require hospitalization or intravenous treatment three or more times in a 12-month period. 1. Sepsis; or 2. Meningitis; or 3. Pneumonia; or 4. Septic arthritis; or 5. Endocarditis; or 6. Sinusitis documented by appropriate medically acceptable imaging. OR L. Any other manifestation(s) of HIV infection, including those listed in 114.08A-K, but without the requisite findings for those listings (for ex- ample, oral candidiasis not meeting the criteria in 114.08F, diarrhea not meeting the criteria in 114.08I), or other manifestation(s) (for example, oral hairy leukoplakia, hepatomegaly), resulting in one of the following: 1. For children from birth to attainment of age 1, at least one of the cri- teria in paragraphs A-E of 112.12; or 2. For children age 1 to attainment of age 3, at least one of the appropri- ate age-group criteria in paragraph B1 of 112.02; or 3. For children age 3 to attainment of age 18, at least two of the appropri- ate age-group criteria in paragraph B2 of 112.02.

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