A. What disorders do we evaluate under the immune system disorders listings?
1. We evaluate 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.
Immune system disorders can cause a deficit in a single organ or body system that results in extreme (that is, very serious)
loss of function. They can also cause lesser degrees of limitations in two or more organs or body systems, and when associated
with symptoms or signs, such as 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 (14.00D).Autoimmune disorders are caused by
dysfunctional immune responses directed against the body’s own tissues, resulting in chronic, multisystem impairments
that differ in clinical manifestations, course, and outcome. They are sometimes referred to as rheumatic diseases, connective
tissue disorders, or collagen vascular disorders. Some of the features of autoimmune disorders in adults differ from the features
of the same disorders in children.
3. Immune deficiency disorders, excluding HIV
infection (14.00E).Immune deficiency disorders are characterized by recurrent or unusual infections that respond poorly
to treatment, and are often associated with complications affecting other parts of the body. Immune deficiency disorders are
classified as either primary (congenital) or acquired. Individuals with immune deficiency disorders also
have an increased risk of malignancies and of having autoimmune disorders.
4. Human immunodeficiency
virus (HIV) infection (14.00F).HIV infection may be characterized by increased susceptibility to opportunistic
infections, cancers, or other conditions, as described in 14.08. B. What information do we need to show that
you have an immune system disorder? Generally, we need your medical history, a report(s) of a physical examination, a
report(s) of laboratory findings, and in some instances, appropriate medically acceptable imaging or tissue biopsy reports
to show that you have an immune system disorder. Therefore, we will make every reasonable effort to obtain your medical history,
medical findings, and results of laboratory tests. We explain the information we need in more detail in the sections below.
1. Appropriate medically acceptable imaging includes,
but is not limited to, angiography, x-ray imaging, computerized axial tomography (CAT scan) or magnetic resonance imaging
(MRI), with or without contrast material, myelography, and radionuclear bone scans. "Appropriate" means that the
technique used is the proper one to support the evaluation and diagnosis of the impairment.
Constitutional symptoms or signs, as used in these listings, means severe fatigue, fever, malaise, or involuntary
weight loss. Severe fatigue means a frequent sense of exhaustion that results in significantly reduced physical activity
or mental function. Malaise means frequent feelings of illness, bodily discomfort, or lack of well-being that result
in significantly reduced physical activity or mental function.
3. Disseminated means
that a condition is spread over a considerable area. The type and extent of the spread will depend on your specific disease.
4. Dysfunction means that one or more of the body regulatory mechanisms are impaired, causing either an
excess or deficiency of immunocompetent cells or their products.
5. Extra-articular means
"other than the joints"; for example, an organ(s) such as the heart, lungs, kidneys, or skin.
6. Inability to ambulate effectively has the same meaning as in 1.00B2b.
Inability to perform fine and gross movements effectively has the same meaning as in 1.00B2c.
8. Major peripheral joints has the same meaning as in 1.00F.
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.
Recurrent means that a condition that previously responded adequately to an appropriate course of treatment returns
after a period of remission or regression. The precise meaning, such as the extent of response or remission and the time periods
involved, will depend on the specific disease or condition you have, the body system affected, the usual course of the disorder
and its treatment, and the other facts of your particular case.
11. Resistant to treatment
means that a condition did not respond adequately to an appropriate course of treatment. Whether a response is adequate
or a course of treatment is appropriate will depend on the specific disease or condition you have, the body system affected,
the usual course of the disorder and its treatment, and the other facts of your particular case.
Severe 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 connection with a finding at the second step of the sequential evaluation processes in §§404.1520,
416.920, and 416.924.
D. How do we document and evaluate the listed autoimmune disorders?
1. Systemic lupus erythematosus (14.02).
a. General.Systemic lupus
erythematosus (SLE) is a chronic inflammatory disease that can affect any organ or body system. It is frequently, but not
always, accompanied by constitutional symptoms or signs (severe fatigue, fever, malaise, involuntary weight loss). Major organ
or body system involvement can include: Respiratory (pleuritis, pneumonitis), cardiovascular (endocarditis, myocarditis, pericarditis,
vasculitis), renal (glomerulonephritis), hematologic (anemia, leukopenia, thrombocytopenia), skin (photosensitivity), neurologic
(seizures), mental (anxiety, fluctuating cognition ("lupus fog"), mood disorders, organic brain syndrome, psychosis),
or immune system disorders (inflammatory arthritis). Immunologically, there is an array of circulating serum autoantibodies
and pro- and anti-coagulant proteins that may occur in a highly variable pattern.
of SLE.Generally, but not always, the medical evidence will show that your SLE satisfies the criteria in the current
"Criteria for the Classification of Systemic Lupus Erythematosus" by the American College of Rheumatology found
in the most recent edition of the Primer on the Rheumatic Diseases published by the Arthritis Foundation.
2. Systemic vasculitis (14.03).
Vasculitis is an inflammation of blood vessels. It may occur acutely in association with adverse drug reactions, certain chronic
infections, and occasionally, malignancies. More often, it is chronic and the cause is unknown. Symptoms vary depending on
which blood vessels are involved. Systemic vasculitis may also be associated with other autoimmune disorders; for example,
SLE or dermatomyositis.
(ii) There are several clinical patterns, including but not limited to
polyarteritis nodosa, Takayasu’s arteritis (aortic arch arteritis), giant cell arteritis (temporal arteritis), and Wegener’s
b. Documentation of systemic vasculitis.Angiography or tissue biopsy
confirms a diagnosis of systemic vasculitis when the disease is suspected clinically. When you have had angiography or tissue
biopsy for systemic vasculitis, we will make every reasonable effort to obtain reports of the results of that procedure. However,
we will not purchase angiography or tissue biopsy.
3. Systemic sclerosis (scleroderma) (14.04).
a. General.Systemic sclerosis (scleroderma) constitutes a spectrum of disease in which thickening of the
skin is the clinical hallmark. Raynaud’s phenomenon, often medically severe and progressive, is present frequently and
may be the peripheral manifestation of a vasospastic abnormality in the heart, lungs, and kidneys. The CREST syndrome (calcinosis,
Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is a variant that may slowly progress
over years to the generalized process, systemic sclerosis.
b. Diffuse cutaneous systemic sclerosis.In
diffuse cutaneous systemic sclerosis (also known as diffuse scleroderma), major organ or systemic involvement can include
the gastrointestinal tract, lungs, heart, kidneys, and muscle in addition to skin or blood vessels. Although arthritis can
occur, joint dysfunction results primarily from soft tissue/cutaneous thickening, fibrosis, and contractures.
c. Localized scleroderma (linear scleroderma and morphea).
scleroderma (linear scleroderma and morphea) is more common in children than in adults. However, this type of scleroderma
can persist into adulthood. To assess the severity of the impairment, we need a description of the extent of involvement of
linear scleroderma and the location of the lesions. For example, linear scleroderma involving the arm but not crossing any
joints is not as functionally limiting as sclerodactyly (scleroderma localized to the fingers). Linear scleroderma of a lower
extremity involving skin thickening and atrophy of underlying muscle or bone can result in contractures and leg length discrepancy.
In such cases, we may evaluate your impairment under the musculoskeletal listings (1.00).
When there is isolated morphea of the face causing facial disfigurement from unilateral hypoplasia of the mandible, maxilla,
zygoma, or orbit, adjudication may be more appropriate under the criteria in the affected body system, such as special senses
and speech (2.00) or mental disorders (12.00).
(iii) Chronic variants of these syndromes include
disseminated morphea, Shulman’s disease (diffuse fasciitis with eosinophilia), and eosinophilia-myalgia syndrome (often
associated with toxins such as toxic oil or contaminated tryptophan), all of which can impose medically severe musculoskeletal
dysfunction and may also lead to restrictive pulmonary disease. We evaluate these variants of the disease under the criteria
in the musculoskeletal listings (1.00) or respiratory system listings (3.00).
of systemic sclerosis (scleroderma).Documentation involves differentiating the clinical features of systemic
clerosis (scleroderma) from other autoimmune disorders. However, there may be an overlap.
and dermatomyositis (14.05).
a. General.Polymyositis and dermatomyositis
are related disorders that are characterized by an inflammatory process in striated muscle, occurring alone or in association
with other autoimmune disorders or malignancy. The most common manifestations are symmetric weakness, and less frequently,
pain and tenderness of the proximal limb-girdle (shoulder or pelvic) musculature. There may also be involvement of the cervical,
cricopharyngeal, esophageal, intercostal, and diaphragmatic muscles.
b. Documentation of polymyositis
and dermatomyositis.Generally, but not always, polymyositis is associated with elevated serum muscle enzymes
(creatine phosphokinase (CPK), aminotransferases, and aldolase), and characteristic abnormalities on electromyography and
muscle biopsy. In dermatomyositis there are characteristic skin findings in addition to the findings of polymyositis. When
you have had electromyography or muscle biopsy for polymyositis or dermatomyositis, we will make every reasonable effort to
obtain reports of the results of that procedure. However, we will not purchase electromyography or muscle biopsy.
c. Additional information about how we evaluate polymyositis and dermatomyositis under the
(i) Weakness of your pelvic girdle muscles that results in your inability to rise
independently from a squatting or sitting position or to climb stairs may be an indication that you are unable to ambulate
effectively. Weakness of your shoulder girdle muscles may result in your inability to perform lifting, carrying, and reaching
overhead, and also may seriously affect your ability to perform activities requiring fine movements. We evaluate these limitations
(ii) We use the malignant neoplastic diseases listings (13.00) to evaluate malignancies
associated with polymyositis or dermatomyositis. We evaluate the involvement of other organs/body systems under the criteria
for the listings in the affected body system.
5. Undifferentiated and mixed connective
tissue disease (14.06).
a. General.This listing includes syndromes with clinical
and immunologic features of several autoimmune disorders, but which do not satisfy the criteria for any of the specific disorders
described. For example, you may have clinical features of SLE and systemic vasculitis, and the serologic (blood test) findings
of rheumatoid arthritis.
b. Documentation of undifferentiated and mixed connective
tissue disease.Undifferentiated connective tissue disease is diagnosed when clinical features and serologic (blood test)
findings, such as rheumatoid factor or antinuclear antibody (consistent with an autoimmune disorder) are present but do not
satisfy the criteria for a specific disease. Mixed connective tissue disease (MCTD) is diagnosed when clinical features and
serologic findings of two or more autoimmune diseases overlap.
6. Inflammatory arthritis (14.09).
a. General.The spectrum of inflammatory arthritis includes a vast array of disorders that differ in cause,
course, and outcome. Clinically, inflammation of major peripheral joints may be the dominant manifestation causing difficulties
with ambulation or fine and gross movements; there may be joint pain, swelling, and tenderness. The arthritis may affect other
joints, or cause less limitation in ambulation or the performance of fine and gross movements. However, in combination with
extra-articular features, including constitutional symptoms or signs (severe fatigue, fever, malaise, involuntary weight loss),
inflammatory arthritis may result in an extreme limitation.
b. Inflammatory arthritis involving
the axial spine (spondyloarthropathy).In adults, inflammatory arthritis involving the axial spine may be associated
with disorders such as:
(i) Reiter’s syndrome;
(iii) Psoriatic arthritis;
(iv) Whipple’s disease;
(v) Behçet’s disease; and
(vi) Inflammatory bowel disease.
c. Inflammatory arthritis involving the peripheral joints.In adults, inflammatoryarthritis involving peripheral
joints may beassociated with disorders such as:
(i) Rheumatoid arthritis;
(iii) Psoriatic arthritis;
deposition disorders (gout andpseudogout);
(v) Lyme disease; and
Inflammatory bowel disease.
d. Documentation of inflammatory arthritis.Generally, but
not always, thediagnosis of inflammatory arthritis is basedon the clinical features and serologic findingsdescribed in the
most recent edition of the Primer on the Rheumatic Diseases publishedby the Arthritis Foundation.
e. How we evaluate inflammatory arthritis under the listings.
severity in 14.09A and14.09C1 is shown by an impairment thatresults in an "extreme" (very serious)limitation. In
14.09A, the criterion is satisfiedwith persistent inflammation or deformity inone major peripheral weight-bearing jointresulting
in the inability to ambulateeffectively (as defined in 14.00C6) or onemajor peripheral joint in each upperextremity resulting
in the inability to performfine and gross movements effectively (asdefined in 14.00C7). In 14.09C1, if you havethe required
ankylosis (fixation) of yourcervical or dorsolumbar spine, we will findthat you have an extreme limitation in yourability
to see in front of you, above you, andto the side. Therefore, inability to ambulateeffectively is implicit in 14.09C1, eventhough
you might not require bilateral upperlimb assistance.
(ii) Listing-level severity is shown in14.09B,
14.09C2, and 14.09D byinflammatory arthritis that involves variouscombinations of complications of one ormore major peripheral
joints or other joints,such as inflammation or deformity, extraarticularfeatures, repeated manifestations,and constitutional
symptoms or signs. Extraarticularimpairments may also meet listingsin other body systems.
Extra-articular features ofinflammatory arthritis may involve any bodysystem; for example: Musculoskeletal (heelenthesopathy),
ophthalmologic (iridocyclitis,keratoconjunctivitis sicca, uveitis),pulmonary (pleuritis, pulmonary fibrosis ornodules, restrictive
lung disease),cardiovascular (aortic valve insufficiency,arrhythmias, coronary arteritis, myocarditis,pericarditis, Raynaud’s
phenomenon,systemic vasculitis), renal (amyloidosis of thekidney), hematologic (chronic anemia,thrombocytopenia), neurologic
(peripheralneuropathy, radiculopathy, spinal cord orcauda equina compression with sensory and motor loss), mental (cognitive
dysfunction, poor memory), and immune system (Felty’s syndrome (hypersplenism with compromised immune competence)).
(iv) If both inflammation and chronic deformities are present, we evaluate your impairment under the criteria of
any appropriate listing.
7. Sjögren’s syndrome (14.10).
(i) Sjögren’s syndrome is an immunomediated disorder
of the exocrine glands. Involvement of the lacrimal and salivary glands is the hallmark feature, resulting in symptoms of
dry eyes and dry mouth, and possible complications, such as corneal damage, blepharitis (eyelid inflammation), dysphagia (difficulty
in swallowing), dental caries, and the inability to speak for extended periods of time. Involvement of the exocrine glands
of the upper airways may result in persistent dry cough.
(ii) Many other organ systems may be
involved, including musculoskeletal (arthritis, myositis), respiratory (interstitial fibrosis), gastrointestinal (dysmotility,
dysphagia, involuntary weight loss), genitourinary (interstitial cystitis, renal tubular acidosis), skin (purpura, vasculitis),
neurologic (central nervous system disorders, cranial and peripheral neuropathies), mental (cognitive dysfunction, poor memory),
and neoplastic (lymphoma). Severe fatigue and malaise are frequently reported. Sjögren’s syndrome may be associated
with other autoimmune disorders (for example, rheumatoid arthritis or SLE); usually the clinical features of the associated
b. Documentation of Sjögren’s syndrome.If you have Sjögren’s
syndrome, the medical evidence will generally, but not always, show that your disease satisfies the criteria in the current
"Criteria for the Classification of Sjögren’s Syndrome" by the American College of Rheumatology found
in the most recent edition of the Primer on the Rheumatic Diseases published by the Arthritis Foundation.
E. How do we document and evaluate immune deficiency disorders, excluding HIV infection?
a. Immune deficiency disorders can be classified as:
(i) Primary (congenital); for example, Xlinked agammaglobulinemia, thymic hypoplasia (DiGeorge syndrome),
severe combined immunodeficiency (SCID), chronic granulomatous disease (CGD), C1 esterase inhibitor deficiency.
(ii) Acquired; for example, medication related.
b. Primary immune deficiency
disorders are seen mainly in children. However, recent advances in the treatment of these disorders have allowed many affected
children to survive well into adulthood. Occasionally, these disorders are first diagnosed in adolescence or adulthood.
2. Documentation of immune deficiency disorders.The medical evidence must include documentation
of the specific type of immune deficiency. Documentation may be by laboratory evidence or by other generally acceptable methods
consistent with the prevailing state of medical knowledge and clinical practice.
deficiency disorders treated by stem cell transplantation.
a. Evaluation in
the first 12 months.If you undergo stem cell transplantation for your immune deficiency disorder, we will consider you
disabled until at least 12 months from the date of the transplant.
b. Evaluation after the
12-month period has elapsed.After the 12-month period has elapsed, we will consider any residuals of your immune
deficiency disorder as well as any residual impairment(s) resulting from the treatment, such as complications arising from:
(i) Graft-versus-host (GVH) disease.
(ii) Immunosuppressant therapy, such as frequent
(iii) Significant deterioration of other organ systems.
Medication-induced immune suppression.Medication effects can result in varying degrees of immune suppression,
but most resolve when the medication is ceased. However, if you are prescribed medication for long-term immune suppression,
such as after an organ transplant, we will evaluate:
a. The frequency and severity of infections.
b. Residuals from the organ transplant itself, after the 12-month period has elapsed.
Significant deterioration of other organ systems.
F. How do we document and evaluate human
immunodeficiency virus (HIV) infection? Any individual with HIV infection, including one with a diagnosis of acquired immune
deficiency syndrome (AIDS), may be found disabled under 14.08 if his or her impairment meets the criteria in that listing
or is medically equivalent to the criteria in that listing.
1. Documentation of HIV infection.The
medical evidence must include documentation of HIV infection. Documentation may be by laboratory evidence or by other generally
acceptable methods consistent with the prevailing state of medical knowledge and clinical practice. When you have had laboratory
testing for HIV infection, we will make every reasonable effort to obtain reports of the results of that testing. However,
we will not purchase laboratory testing to establish whether you have HIV infection.
documentation of HIV infection.A definitive diagnosis of HIV infection is documented by one or more of the following
(i) HIV antibody tests. HIV antibodies are usually first detected by an ELISA
screening test performed on serum. Because the ELISA can yield false positive results, confirmation is required using a more
definitive test, such as a Western blot or an immunofluorescence assay.
(ii) Positive "viral
load" (VL) tests. These tests are normally used to quantitate the amount of the virus present but also document HIV infection.
Such tests include the quantitative plasma HIV RNA, quantitative plasma HIV branched DNA, and reverse transcriptase-polymerase
chain reaction (RT-PCR).
(iii) HIV DNA detection by polymerase chain reaction (PCR).
(iv) A specimen that contains HIV antigen (for example, serum specimen, lymphocyte culture, or cerebrospinal fluid).
(v) A positive viral culture for HIV from peripheral blood mononuclear cells (PBMC).
Other tests that are highly specific for detection of HIV and that are consistent with the prevailing state of medical knowledge.
b. Other acceptable documentation of HIV infection. We may also document HIV infection without the definitive
laboratory evidence described in 14.00F1a, provided that such documentation is consistent with the prevailing state of medical
knowledge and clinical practice and is consistent with the other evidence in your case record. If no definitive laboratory
evidence is available, we may document HIV infection by the medical history, clinical and laboratory findings, and diagnosis(es)
indicated in the medical evidence. For example, we will accept a diagnosis of HIV infection without definitive laboratory
evidence 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, medical findings, and results of testing.
tests.Individuals who have HIV infection or other disorders of the immune system may have tests showing a reduction of
either the absolute count or the percentage of their T-helper lymphocytes (CD4 cells). The extent of immune suppression correlates
with the level or rate of decline of the CD4 count. Generally, when the CD4 count is 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 definitive diagnosis of an opportunistic infection(s). However, a reduced CD4 count
alone does not document the severity or functional consequences of HIV infection.
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 acceptable methods consistent
with the prevailing state of medical knowledge and clinical practice.
a. Definitive 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 specific laboratory evidence of an opportunistic
disease or other condition whenever this information is available. If a histologic or other test has been performed, the evidence
should include a copy of the appropriate report. If we cannot obtain the report, the summary of hospitalization or a report
from the treating source should include details of the findings and results of the diagnostic studies (including appropriate
medically acceptable imaging studies) or microscopic examination of the appropriate tissues or body fluids.
b. Other acceptable documentation of the manifestations of HIV infection. We may also document manifestations
of HIV infection without the definitive laboratory evidence described in 14.00F3a, provided that such documentation is consistent
with the prevailing state of medical knowledge and clinical practice and is consistent with the other evidence in your case
record. 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 manifestations 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. Supportive 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.
Documentation of Cytomegalovirus (CMV) disease (14.08D) may present special problems because definitive diagnosis
(except for chorioretinitis, 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 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 commonly performed. This condition is usually
diagnosed presumptively based on symptoms or signs of fever, headache, focal neurologic deficits, seizures, typical lesions
on brain imaging, and a positive serology test.
(iv) Candidiasis of the esophagus (also known
as Candida esophagitis) 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
4. HIV infection manifestations specific to women.
General. Most women with severe immunosuppression secondary to HIV infection exhibit the typical opportunistic infections
and other conditions, such as PCP, Candida esophagitis, wasting syndrome, cryptococcosis, and toxoplasmosis. However,
HIV infection may have different manifestations in women than in men. Adjudicators must carefully scrutinize the medical evidence
and be alert to the variety of medical conditions specific to, or common in, women with HIV infection that may affect their
ability to function in the workplace.
b. Additional considerations for evaluating HIV infection
in women. Many of these manifestations (for example, vulvovaginal candidiasis, pelvic inflammatory disease) occur in
women with or without HIV infection, but can be more severe or resistant to treatment, or occur more frequently in a woman
whose immune system is suppressed. Therefore, when evaluating the claim of a woman with HIV infection, it is important to
consider gynecologic and other problems specific to women, including any associated symptoms (for example, pelvic pain), in
assessing the severity of the impairment and resulting functional limitations. We may evaluate manifestations of HIV infection
in women under the specific criteria (for example, cervical cancer under 14.08E), under an applicable general category (for
example, pelvic inflammatory disease under 14.08A4) or, in appropriate cases, under 14.08K.
Involuntary weight loss. For purposes of 14.08H, an involuntary weight loss of at least 10 percent of baseline is
always considered "significant." Loss of less than 10 percent may or may not be significant, depending on the individual’s
baseline weight and body habitus. For example, a 7-pound weight loss in a 100-pound woman who is 63 inches tall might be considered
significant; but a 14-pound weight loss in a 200-pound woman who is the same height might not be significant. 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 evaluating your autoimmune disorder, immune deficiency disorder, or HIV infection?
1. General.If your impairment does not otherwise meet the requirements of a listing, we will consider your
medical treatment in terms of its effectiveness in improving the signs, symptoms, and laboratory abnormalities of your specific
immune system disorder or its manifestations, and in terms of any side effects that limit your functioning. We will make every
reasonable effort to obtain a specific description of the treatment you receive (including surgery) for your immune system
disorder. We consider:
a. The effects of medications you take.
Adverse side effects (acute and chronic).
c. The intrusiveness and complexity of your treatment
(for example, the dosing schedule, need for injections).
d. The effect of treatment on your mental
functioning (for example, cognitive changes, mood disturbance).
e. Variability of your response
to treatment (see 14.00G2).
f. The interactive and cumulative effects of your treatments. For
example, many individuals with immune system disorders receive treatment both for their immune system disorders and for the
manifestations of the disorders or co-occurring impairments, such as treatment for HIV infection and hepatitis C. The interactive
and cumulative effects of these treatments may be greater than the effects of each treatment considered separately.
g. The duration of your treatment.
h. Any other aspects of treatment that may interfere
with your ability to function.
2. Variability of your response to treatment.Your response
to treatment and the adverse or beneficial consequences of your treatment may vary widely. The effects of your treatment may
be temporary or long term. For example, some individuals may show an initial positive response to a drug or combination of
drugs followed by a decrease in effectiveness. When we evaluate your response to treatment and how your treatment may affect
you, we consider such factors as disease activity before treatment, requirements for changes in therapeutic 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 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 evaluate the effects
of treatment for autoimmune disorders on your ability to function.Some medications may have acute or long-term side effects.
When we consider the effects of corticosteroids or other treatments for autoimmune disorders on your ability to function,
we consider the factors in 14.00G1 and 14.00G2. Long-term corticosteroid treatment can cause ischemic necrosis of bone, posterior
subcapsular cataract, weight gain, glucose intolerance, increased susceptibility to infection, and osteoporosis that may result
in a loss of function. In addition, medications used in the treatment of autoimmune disorders may also have effects on mental
functioning, including cognition (for example, memory), concentration, and mood.
4. How we
evaluate the effects of treatment for immune deficiency disorders, excluding HIV infection, on your ability to function.When
we consider the effects of your treatment for your immune deficiency disorder on your ability to function, we consider the
factors in 14.00G1 and 14.00G2. A frequent need for treatment such as intravenous immunoglobulin and gamma interferon therapy
can be intrusive and interfere with your ability to work. 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, shortness of breath, or limitations in mental function including cognition (for example, memory), concentration, and
5. How we evaluate the effects of treatment for HIV infection on your ability to function.
a. General.When we consider the effects of antiretroviral drugs (including the effects
of highly active antiretroviral therapy (HAART)) and the effects of treatments for the manifestations of HIV infection on
your ability to function, we consider the factors in 14.00G1 and 14.00G2. Side effects of antiretroviral drugs include, but
are not limited to: Bone marrow suppression, pancreatitis, gastrointestinal intolerance (nausea, vomiting, diarrhea), neuropathy,
rash, hepatotoxicity, lipodystrophy (fat redistribution, 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
interruption (STI, also called a "drug holiday") is a treatment practice during which your treating source advises
you to stop taking your medications temporarily. An STI in itself does not imply that your medical condition has improved;
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 treatment or draw inferences about the severity of your impairment on this fact alone.
We will consider why your treating source has prescribed or recommended an STI and all the other information in your case
record when we determine the severity of your impairment.
6. When there is no record of ongoing
treatment.If you have not received ongoing treatment or have not had an ongoing relationship with the medical community
despite the existence of a severe impairment(s), we will evaluate the medical severity and duration of your immune system
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 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 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, severe 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 findings
showing the existence of a medically determinable impairment(s) that could reasonably be expected to produce the symptoms.
If you have such an impairment(s), we will evaluate the intensity, persistence, and functional effects of your symptoms using
the rules throughout 14.00 and in our other regulations. See §§404.1528, 404.1529, 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 evaluating 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 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
3. As used in these listings, "repeated" means that the manifestations occur on an average
of three times a year, or once every 4 months, each lasting 2 weeks or more; or the manifestations do not last for 2 weeks
but occur substantially more frequently than three times in a year or once every 4 months; or they occur less frequently than
an average of three times a year or once every 4 months but last substantially longer than 2 weeks. 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 disorder must result in a "marked" level of limitation in one of three general areas of functioning: Activities
of daily living, social functioning, or difficulties in completing tasks due to deficiencies in concentration, persistence,
or pace. Functional limitation may result from the impact of the disease process itself on your mental functioning, physical
functioning, or both your mental and physical functioning. This could result from persistent or intermittent symptoms, such
as depression, 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 14.00G).
5. When "marked" is used as a standard for measuring
the degree of functional limitation, it means more than moderate but less than extreme. We do not define "marked"
by a specific number of different activities of daily living in which your functioning is impaired, different behaviors in
which your social functioning is impaired, or tasks that you are able to complete, but by the nature and overall degree of
interference with your functioning. You may have a marked limitation when several activities or functions are impaired, or
even when only one is impaired. Also, you need not be totally precluded from performing an activity to have a marked limitation,
as long as the degree of limitation seriously interferes with your ability to function independently, appropriately, and effectively.
The term "marked" does not imply that you must be confined to bed, hospitalized, or in a nursing home.
of daily living include, but are not limited to, such activities as doing household chores, grooming and hygiene, using
a post office, taking public transportation, or paying bills. We will find that you have a "marked" limitation of
activities of daily living if you have a serious limitation in your ability to maintain a household or take public transportation
because of symptoms, such as pain, severe fatigue, anxiety, or difficulty concentrating, caused by your immune system disorder
(including manifestations of the disorder) or its treatment, even if you are able to perform some self-care activities.
7. Social functioning includes the capacity to interact independently, appropriately, effectively, and on a sustained
basis with others. It includes the ability to communicate effectively with others. We will find that you have a "marked"
limitation in maintaining social functioning if you have a serious limitation in social interaction on a sustained basis because
of symptoms, such as pain, severe fatigue, anxiety, or difficulty concentrating, or a pattern of exacerbation and remission,
caused by your immune system disorder (including manifestations of the disorder) or its treatment, even if you are able to
communicate with close friends or relatives.
8. Completing tasks in a timely manner involves the ability to
sustain concentration, persistence, or pace to permit timely completion of tasks commonly found in work settings. We will
find that you have a "marked" limitation in completing tasks if you have a serious limitation in your ability to
sustain concentration or pace adequate to complete work-related tasks because of symptoms, such as pain, severe fatigue, anxiety,
or difficulty concentrating, caused by your immune system disorder (including manifestations of the disorder) or its treatment,
even if you are able to do some routine activities of daily living.J. How do we evaluate your immune system disorder when
it does not meet one of these listings?
1. These listings are only examples of immune system
disorders that we consider severe enough to prevent you from doing any gainful activity. If your impairment(s) does not meet
the criteria of any of these listings, we must also consider whether you have an impairment(s) that satisfies the criteria
of a listing in another body system.
2. Individuals with immune system disorders, including HIV
infection, may manifest signs or symptoms of a mental impairment or of another physical impairment. We may evaluate these
impairments under any affected body system. For example, we will evaluate:
involvement, such as surgical reconstruction of a joint, under 1.00.
b. Ocular involvement, such
as dry eye, under 2.00.
c. Respiratory impairments, such as pleuritis, under 3.00.
d. Cardiovascular impairments, such as cardiomyopathy, under 4.00.
e. Digestive impairments,
such as hepatitis (including hepatitis C) or weight loss as a result of HIV infection that affects the digestive system, under
f. Genitourinary impairments, such as nephropathy, under 6.00.
g. Hematologic abnormalities,
such as anemia, granulocytopenia, and thrombocytopenia, under 7.00.
h. Skin impairments, such
as persistent fungal and other infectious skin eruptions, and photosensitivity, under 8.00.
Neurologic impairments, such as neuropathy or seizures, under 11.00.
j. Mental disorders, such
as depression, anxiety, or cognitive deficits, under 12.00.
k. Allergic disorders, such as asthma
or atopic dermatitis, under 3.00 or 8.00 or under the criteria in another affected body system.
Syphilis or neurosyphilis under the criteria for the affected body system; for example, 2.00 Special senses and speech, 4.00
Cardiovascular system, or 11.00 Neurological.
3. If you have a severe medically determinable impairment(s)
that does not meet a listing, we will determine whether your impairment(s) medically equals a listing. (See §§404.1526
and 416.926.) If it does not, you may or may not have the residual functional capacity to engage in substantial gainful activity.
Therefore, we proceed to the fourth, and if necessary, the fifth steps of the sequential evaluation process in §§404.1520
and 416.920. We use the rules in §§404.1594, 416.994, and 416.994a as appropriate, when we decide whether you continue
to be disabled.