DI 34134.007 Immune Listings from 05/24/02 – 06/15/08
14.00 Immune System
A. Listed disorders include impairments involving deficiency of one or more components
of the immune system (i.e., antibody-producing B cells; a number of different types
of cells associated with cell-mediated immunity including T-lymphocytes, macrophages
and monocytes; and components of the complement system).
B. Dysregulation of the immune system may result in the development of a connective
tissue disorder. Connective tissue disorders include several chronic multisystem disorders
that differ in their clinical manifestation, course, and outcome. They generally evolve
and persist for months or years, may result in loss of functional abilities, and may
require long-term, repeated evaluation and management.
The documentation needed to establish the existence of a connective tissue disorder
is medical history, physical examination, selected laboratory studies, appropriate
medically acceptable imaging and, in some instances, tissue biopsy. Medically acceptable
imaging includes, but is not limited to,
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.
However, the Social Security Administration will not purchase diagnostic tests or
procedures that may involve significant risk, such as biopsies or angiograms. Generally,
the existing medical evidence will contain this information.
A longitudinal clinical record of at least 3 months demonstrating active disease despite
prescribed treatment during this period with the expectation that the disease will
remain active fir 12 months is necessary for assessment of severity and duration of
To permit appropriate application of a listing, the specific diagnostic features that
should be documented in the clinical record for each of the disorders are summarized
for systemic lupus erythematosus (SLE), systemic vasculitis, systemic sclerosis and
scleroderma, polymyositis or dermatomyositis, undifferentiated connective tissue disorders,
and the inflammatory arthritides.
In addition to the limitations caused by the connective tissue disorder per se , the chronic adverse effects of treatment (e.g., corticosteroid-related ischemic
necrosis of bone) may result in functional loss.
These disorders may preclude performance of any gainful activity by reason of serious
loss of function because of disease affecting a single organ or body system, or lesser
degrees of functional loss because of disease affecting two or more organs/body systems
associated with significant constitutional symptoms and signs of severe fatigue, fever,
malaise, weight loss, and joint pain and stiffness. We use the term "severe" in these
listings to describe medical severity; 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 DI 22001.000 and DI 25201.005 .
1. Systemic lupus erythematosus (14.02)—This disease is characterized clinically by
constitutional symptoms and signs (e.g., fever, fatigability, malaise, weight loss),
multisystem involvement and, frequently, anemia, leukopenia, or thrombocytopenia.
Immunologically, an array of circulating serum auto-antibodies can occur, but are
highly variable in pattern. Generally the medical evidence will show that patients
with this disease will fulfill The 1982 Revised Criteria for the Classification of
Systemic Lupus Erythematosus of the American College of Rheumatology. (Tan, E.M.,
et al., Arthritis Rheum.25: 11271-1277, 1982).
2. Systemic vasculitis (14.03)—This disease occurs acutely in association with adverse
drug reactions, certain chronic infections and, occasionally, malignancies. More often
it is idiopathic and chronic. There are several clinical patterns, including classical
polyarteritis nodosa, aortic arch arteritis, giant cell arteritis, Wgener's granulomatosis,
and vasculitis associated with other connective tissue disorders (e.g., rheumatoid
arthritis, SLE, Sjögrens syndrome, cryoglobulinemia). Cutaneous vasculitis may or
may not be associated with systemic involvement and the patterns of vascular and ischemic
involvement are highly variable. The diagnosis is confirmed by angiography or tissue
biopsy when the disease is suspected clinically. Most patients who are stated to have
this disease will have the results of the confirmatory angiogram or biopsy in their
3. Systemic sclerosis and scleroderma (14.04)—These disorders constitute a spectrum
of disease in which thickening of the skin is the clinical hallmark. Raynaud's phenomena,
often severe and progressive, are especially frequent and may be the peripheral manifestation
of a generalized vasospastic abnormality in the heart, lungs, and kidneys. The CREST
syndrome (calcinosis, Raynaud's phenomena, esophageal dysmotility, sclerodactyly,
telangiectasia) is a variant that may slowly progress to the generalized process,
systemic sclerosis, over years. In addition to skin and blood vessels, the major organ/body
system involvement includes the gastrointestinal tract, lungs, heart, kidneys, and
muscle. Although arthritis can occur, joint dysfunction results primarily from soft
tissue/cutaneous thickening, fibrosis, and contractures.
4. Polymyositis or dermatomyositis (14.05)—This disorder is primarily an inflammatory
process in striated muscle, which can occur alone or in association with other connective
tissue disorders or malignancy. Weakness, and less frequently, pain and tenderness
of the proximal limb-girdle musculature are the cardinal manifestations. Involvement
of the cervical muscles, the cricopharyngeals, the intercostals, and diaphragm may
occur in those with listing-level disease. Weakness of the pelvic girdle, as contemplated
in Listing 14.05, may result in significant difficulty climbing stairs or rising from
a chair without use of the arms. Proximal limb weakness in the upper extremities may
result in inability to life objects and interference with dressing and combing hair.
Weakness of anterior neck flexors may impair the ability to life the head from the
pillow in bed. The diagnosis is supported by elevated serum muscle enzymes (creatine
phosphokinase (CPK), aminotransferases, aldolase), characteristic abnormalities on
electromyography, and myositis on muscle biopsy.
5. Undifferentiated connective tissue disorder (14.06)—This listing includes syndromes
with clinical and immunologic features of several connective tissue disorders, but
that do not satisfy the criteria for any of the disorders described: for instance,
the individual may have clinical features of systemic lupus erythematosus and systemic
vasculitis and the serologic findings of rheumatoid arthritis. It also includes overlap
syndromes with clinical features of more than one established connective tissue disorder.
For example, the individual may have features of both rheumatoid arthritis and scleroderma.
The correct designation of this disorder is important for assessment of prognosis.
6. Inflammatory arthritis (14.09) includes a vast array of disorders that differ in
cause, course, and outcome. For example, inflammatory spondyloarthropathies include
ankylosing spondylitis, Reiter's syndrome and other reactive arthropathies, psoriatic
arthropathy, Behçet's disease, and Whipple's disease, as well as undifferentiated
spondylitis. Inflammatory arthritis of peripheral joints likewise comprises many disorders,
including rheumatoid arthritis, Sjögren's syndrome, psoriatic arthritis, crystal deposition
disorders, and Lyme disease. Clinically, inflammation of major joints may be the dominant
problem causing difficulties with ambulation or fine and gross movements, or the arthritis
may involve other joints or cause less restriction of ambulation or other movements
but be complicated by extra-articular features that cumulatively result in serious
functional deficit. When persistent deformity without ongoing inflammation is the
dominant feature of the impairment, it should be evaluated under 1.02, or, if there
has been surgical reconstruction, 1.03.
a. In 14.09A, the term major joints refers to the major peripheral joints, which are the hip, knee, shoulder, elbow,
wrist-hand, and ankle-foot, as opposed to other peripheral joints (e.g., the joints
of the hand or forefoot) or axial joints (i.e., the joints of the spine.) The wrist
and hand are considered together as one major joint, as are the ankle and foot. Since
only the ankle joint, which consists of the juncture of the bones of the lower leg
(tibia and fibula) with the hindfoot (tarsal bones), but not the forefoot, is crucial
to weight bearing, the ankle and foot are considered separately in evaluating weight
b. The terms inability to ambulate effectively and inability to perform fine and gross movements effectively in 14.09A have the same meaning as in 1.00B2b and 1.00B2c and must have lasted, or
be expected to last, for at least 12 months.
c. Inability to ambulate effectively is implicit in 14.09B. Even though individuals
who demonstrate the findings of 14.09B will not ordinarily require bilateral upper
limb assistance, the required ankylosis of the cervical or dorsolumbar spine will
result in an extreme loss of the ability to see ahead, above, and to the side.
d. As in 14.02 through 14.06, extra-articular features of an inflammatory arthritis
may satisfy the criteria for a listing in an involved extra-articular body system.
Such impairments may be found to meet a criterion of 14.09C. Extra-articular impairments
of lesser severity should be evaluated under 14.09D and 14.09E. Commonly occurring
extra-articular impairments include keratoconjunctivitis sicca, uveitis, iridocyclitis,
pleuritis, pulmonary fibrosis or nodules, restrictive lung disease, pericarditis,
myocarditis, cardiac arrhythmias, aortic valve insufficiency, coronary arteritis,
Raynaud's phenomena, systemic vasculitis, amyloidosis of the kidney, chronic anemia,
thrombocytopenia, hypersplenism with compromised immune competence (Felty's syndrome),
peripheral neuropathy, radiculopathy, spinal cord or cauda equina compression with
sensory and motor loss, and heel enthesopathy with functionally limiting pain.
e. The fact that an individual is dependent on steroids, or any other drug, for the
control of inflammatory arthritis is, in and of itself, insufficient to find disability.
Advances in the treatment of inflammatory connective tissue disease and in the administration
of steroids for its treatment have corrected some of the previously disabling consequences
of continuous steroid use. Therefore, each case must be evaluated on its own merits,
taking into consideration the severity of the underlying impairment and any adverse
effects of treatment.
C. Allergic disorders (e.g., asthma or atopic dermatitis) are discussed and evaluated
under the appropriate listing of the affected body system.
D. Human immunodeficiency virus (HIV) infection.
1. HIV infection is caused by a specific retrovirus and may be characterized by susceptibility
to one or more opportunistic diseases, cancers, or other conditions, as described
in 14.08. Any individual with HIV infection, including one with a diagnosis of acquired
immunodeficiency syndrome (AIDS), may be found disabled under this listing if his
or her impairment meets any of the criteria in 14.08 or is of equivalent severity
to any impairment in 14.08.
2. Definitions. In 14.08, the terms “resistant to treatment,” “recurrent,” and “disseminated” have the same general meaning as used by the medical community. The precise meaning
of any of these terms will depend upon the specific disease or condition in question,
the body system affected, the usual course of the disorder and its treatment, and
the other circumstances of the case.
“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 appropriate, will depend
on the facts of the particular case.
“Recurrent” means that a condition that responded adequately to an appropriate course of treatment
has returned after a period of remission or regression. The extent of response (or
remission) and the time periods involved will depend on the facts of the particular
“Disseminated” means that a condition is spread widely over a considerable area or body system(s).
The type and extent of the spread will depend on the specific disease.
As used in 14.08I, “significant involuntary weight loss” does not correspond to a specific minimum amount or percentage of weight loss. Although,
for purposes of this listing, 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 female who is 63 inches tall might
be considered significant; but a 14-pound weight loss in a 200-pound female who is
the same height might not be significant.)
3. 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
a. Documentation of HIV infection by definitive diagnosis. A definitive diagnosis
of HIV infection is documented by one or more of the following laboratory tests:
i. A serum specimen that contains HIV antibodies. HIV antibodies are usually detected
by a screening test. The most commonly used screening test is the ELISA. Although
this test is highly sensitive, it may yield false positive results. Therefore, positive
results from an ELISA must be confirmed by a more definitive test (e.g., Western blot,
ii. A specimen that contains HIV antigen (e.g., serum specimen, lymphocyte culture,
or cerebrospinal fluid (CSF) specimen).
iii. Other test(s) that are highly specific for detection of HIV (e.g., polymerase
chain reaction (PCR)), or that are acceptable methods of detection consistent with
the prevailing state of medical knowledge.
When laboratory testing for HIV infection has been performed, every reasonable effort
must be made to obtain reports of the results of that testing.
Individuals who have HIV infection or other disorders of the immune system may undergo
tests to determine T-helper lymphocyte (CD4) counts. The extent of immune depression
correlates with the level or rate of decline of the CD4 count. In general, when the
CD4 count is 200/mm3 or less (14 percent or less), the susceptibility to opportunistic disease is considerably
increased. However, a reduced CD4 count alone does not establish a definitive diagnosis
of HIV infection, or document the severity or functional effects of HIV infection.
b. Other acceptable documentation of HIV infection.
HIV infection may also be documented without the definitive laboratory evidence described
in paragraph a, provided that such documentation is consistent with the prevailing
state of medical knowledge and clinical practice and is consistent with the other
evidence. If no definitive laboratory evidence is available, HIV infection may be
documented by the medical history, clinical and laboratory findings, and diagnosis(es)
indicated in the medical evidence. For example, a diagnosis of HIV infection will
be accepted without definitive laboratory evidence if the individual has an opportunistic
disease (e.g., toxoplasmosis of the brain, pneumocystis carinii pneumonia (PCP)) predictive
of a defect in cell-mediated immunity, and there is no other known cause of diminished
resistance to that disease (e.g., long-term steroid treatment, lymphoma). In such
cases, every reasonable effort must be made to obtain full details of the history,
medical findings, and results of testing.
4. 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 by other generally acceptable methods consistent with
the prevailing state of medical knowledge and clinical practice.
a. Documentation of the manifestations of HIV infection by definitive diagnosis.
The definitive method of diagnosing opportunistic diseases or conditions that are
manifestations of HIV infection is by culture, serological test, or microscopic examination
of biopsied tissue or other material (e.g., bronchial washings). Therefore, every
reasonable effort must be made to obtain specific laboratory evidence of an opportunistic
disease or other condition whenever this information is available. If a histological
or other test has been performed, the evidence should include a copy of the appropriate
report. If the report is not obtainable, the summary of hospitalization or a report
from the treating source should include details of the findings and results of the
diagnostic studies (including radiographic studies) or microscopic examination of
the appropriate tissues or body fluids.
Although a reduced CD4 lymphocyte count may show that there is an increased susceptibility
to opportunistic infections and diseases (see 14.00D3a, above), that alone does not
establish the presence, severity, or functional effects of a manifestation of HIV
b. Other acceptable documentation of the manifestations of HIV infection.
Manifestations of HIV infection may also be documented without the definitive laboratory
evidence described in paragraph a, provided that such documentation is consistent
with the prevailing state of medical knowledge and clinical practice and is consistent
with the other evidence. If no definitive laboratory evidence is available, manifestations
of HIV infection may be documented by medical history, clinical and laboratory findings,
and diagnosis(es) indicated in the medical evidence. In such cases, every reasonable
effort must be made to obtain full details of the history, medical findings, and results
Documentation of cytomegalovirus (CMV) disease (14.08D) presents special problems
because diagnosis requires identification of viral inclusion bodies or a positive
culture from the affected organ, and the absence of any other infectious agent. A
positive serology test identifies infection with the virus, but does not confirm a
disease process. With the exception of chorioretinitis (which may be diagnosed by
an ophthalmologist), documentation of CMV disease requires confirmation by biopsy
or other generally acceptable methods consistent with the prevailing state of medical
knowledge and clinical practice.
5. Manifestations specific to women. Most women with severe immunosuppression secondary
to HIV infection exhibit the typical opportunistic infections and other conditions,
such as pneumocystis carinii pneumonia (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.
Many of these manifestations (e.g. 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 (e.g., pelvic pain), in assessing the severity of the impairment and resulting
functional limitations. Manifestations of HIV infection in women may be evaluated
under the specific criteria (e.g., cervical cancer under 14.08E), under an applicable
general category (e.g., pelvic inflammatory disease under 14.08A5) or, in appropriate
cases, under 14.08N.
6. Evaluation. The criteria in 14.08 do not describe the full spectrum of diseases
or conditions manifested by individuals with HIV infection. As in any case, consideration
must be given to whether an individual's impairment(s) meets or equals in severity
any other listing in appendix 1 of subpart P (e.g., a neoplastic disorder listed in
13.00ff). Although 14.08 includes cross-references to other listings for the more
common manifestations of HIV infection, other listings may apply.
In addition, the impact of all impairments, whether or not related to HIV infection,
must be considered. For example, individuals with HIV infection may manifest signs
and symptoms of a mental impairment (e.g., anxiety, depression), or of another physical
impairment. Medical evidence should include documentation of all physical and mental
impairments, and the impairment(s) should be evaluated not only under the relevant
listing(s) in 14.08, but under any other appropriate listing(s).
It is also important to remember that individuals with HIV infection, like all other
individuals, are evaluated under the full five-step sequential evaluation process
described in DI 22001.001 ff. If an individual with HIV infection is working and engaging in substantial gainful
activity (SGA), or does not have a severe impairment, the case will be decided at
the first or second step of the sequential evaluation process, and does not require
evaluation under these listings. For an individual with HIV infection who is not engaging
in SGA and has a severe impairment, but whose impairment(s) does not meet or equal
in severity the criteria of a listing, evaluation must proceed through the final steps
of the sequential evaluation process (or, as appropriate, the steps in the medical
improvement review standard) before any conclusion can be reached on the issue of
7. Effect of treatment. Medical treatment must be considered in terms of its effectiveness
in ameliorating the signs, symptoms, and laboratory abnormalities of the specific
disorder, or of the HIV infection itself (e.g. antiretroviral agents) and in terms
of any side effects of treatment that may further impair the individual.
Response to treatment and adverse or beneficial consequences of treatment may vary
widely. For example, an individual with HIV infection who develops pneumonia or tuberculosis
may respond to the same antibiotic regimen used in treating individuals without HIV
infection, but another individual with HIV infection may not respond to the same regimen.
Therefore, each case must be considered on an individual basis, along with the effects
of treatment on the individual's ability to function.
A specific description of the drugs or treatment given (including surgery), dosage,
frequency of administration, and a description of the complications or response to
treatment should be obtained. The effects of treatment may be temporary or long-term.
As such, the decision regarding the impact of treatment should be based on a sufficient
period of treatment to permit proper consideration.
8. Functional criteria. Paragraph N of 14.08 establishes standards for evaluating
manifestations of HIV infection that do not meet the requirements listed in 14.08A-M.
Paragraph N is applicable for manifestations that are not listed in 14.08A-M, as well
as those listed in 14.08A-M that do not meet the criteria of any of the rules in 14.08A-M.
For individuals with HIV infection evaluated under 14.08N, listing-level severity
will be assessed in terms of the functional limitations imposed by the impairment.
The full impact of signs, symptoms, and laboratory findings on the claimant's ability
to function must be considered. Important factors to be considered in evaluating the
functioning of individuals with HIV infection include, but are not limited to: symptoms,
such as fatigue and pain; characteristics of the illness, such as the frequency and
duration of manifestations or periods of exacerbation and remission in the disease
course; and the functional impact of treatment for the disease, including the side
effects of medication.
As used in 14.08N, “repeated” means that the conditions occur on an average of 3 times a year, or once every 4
months, each lasting 2 weeks or more; or the conditions do not last for 2 weeks but
occur substantially more frequently than 3 times in a year or once every 4 months;
or they occur less often than an average of 3 times a year or once every 4 months
but last substantially longer than 2 weeks.
To meet the criteria in 14.08N, an individual with HIV infection must demonstrate
a marked level of restriction in one of three general areas of functioning: activities
of daily living; social functioning; and difficulties in completing tasks due to deficiencies
in concentration, persistence, or pace. Functional restrictions may result from the
impact of the disease process itself on mental or physical functioning, or both. This
could result from extended or intermittent symptoms, such as depression, fatigue,
or pain, resulting in a limitation of the ability to concentrate, to persevere at
a task, or to perform the task at an acceptable rate of speed. Limitations may also
result from the side effects of medication.
When “marked” is used as a standard for measuring the degree of functional limitation, it means
more than moderate, but less than extreme. A marked limitation does not represent
a quantitative measure of the individual's ability to do an activity for a certain
percentage of the time. A marked limitation may be present when several activities
or functions are impaired or even when only one is impaired. However, an individual
need not be totally precluded from performing an activity to have a marked limitation,
as long as the degree of limitation is such as to seriously interfere with the ability
to function independently, appropriately, and effectively. The term “marked” does not imply that the impaired individual is confined to bed, hospitalized, or
in a nursing home.
Activities 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,
and paying bills. An individual with HIV infection who, because of symptoms such as
pain imposed by the illness or its treatment, is not able to maintain a household
or take public transportation on a sustained basis or without assistance (even though
he or she is able to perform some self-care activities) would have marked limitation
of activities of daily living.
Social functioning includes the capacity to interact appropriately and communicate
effectively with others. An individual with HIV infection who, because of symptoms
or a pattern of exacerbation and remission caused by the illness or its treatment,
cannot engage in social interaction on a sustained basis (even though he or she is
able to communicate with close friends or relatives) would have marked difficulty
maintaining social functioning.
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.
An individual with HIV infection who, because of HIV-related fatigue or other symptoms,
is unable to sustain concentration or pace adequate to complete simple work-related
tasks (even though he or she is able to do routine activities of daily living) would
have marked difficulty completing tasks.
14.01 Category of Impairments, Immune System
14.02 Systemic lupus erythematosus. Documented as described in 14.00B1, with:
A. One of the following:
1. Joint involvement, as described under the criteria in 1.00; or
2. Muscle involvement, as described under the criteria in 14.05; or
3. Ocular involvement, as described under the criteria in 2.00 ff.; or
4. Respiratory involvement, as described under the criteria in 3.00 ff.; or
5. Cardiovascular involvement, as described under the criteria in 4.00 ff. or 14.04D;
6. Digestive involvement, as described under the criteria in 5.00 ff.; or
7. Renal involvement, as described under the criteria in 6.00 ff.; or
8. Hematologic involvement, as described under the criteria in 7.00 ff.; or9
9. Skin involvement, as described under the criteria in 8.00 ff.; or
10. Neurological involvement, as described under the criteria in 11.00 ff.; or
11. Mental involvement, as described under the criteria in 12.00 ff.
B. Lesser involvement of two or more organs/body systems listed in paragraph A, with
significant, documented, constitutional symptoms and signs of severe fatigue, fever,
malaise, and weight loss. At least one of the organs /body systems must be involved
to at least a moderate level of severity.
14.03 Systemic vasculitis Documented as described in 14.00B2, including documentation by angiography or tissue
biopsy, with: .
A. Involvement of a single organ or body system, as described under the criteria in
B. Lesser involvement of two or more organs/body systems listed in 14.02A, with significant,
documented, constitutional symptoms and signs of severe fatigue, fever, malaise, and
weight loss. At least one of the organs/ body systems must be involved to at least
a moderate level of severity.
14.04 Systemic sclerosis and scleroderma Documented as described in 14.00B3, with:
A. One of the following:
1. Muscle involvement, as described under the criteria in 14.05; or
2. Respiratory involvement, as described under the criteria in 3.00 ff.; or
3. Cardiovascular involvement, as described under the criteria in 4.00 ff.; or
4. Digestive involvement, as described under the criteria in 5.00 ff.; or
5. Renal involvement, as described under the criteria in 6.00 ff.
B. Lesser involvement of two or more organs/body systems listed in paragraph A, with
significant, documented, constitutional symptoms and signs of severe fatigue, fever,
malaise, and weight loss. At least one of the organs /body systems must be involved
to at least a moderate level of severity.
C. Generalized scleroderma with digital contractures.
D. Severe Raynaud's phenomena, characterized by digital ulcerations, ischemia, or
14.05 Polymyositis or dermatomyositis. Documented as described in 14.00B4, with:
A. Severe proximal limb-girdle (shoulder and/or pelvic) muscle weakness, as described
B. Less severe limb-girdle muscle weakness than in 14.05A, associated with cervical
muscle weakness and one of the following to at least a moderate level of severity:
1. Impaired swallowing with dysphagia and episodes of aspiration due to cricopharyngeal
2. Impaired respiration due to intercostal and diaphragmatic muscle weakness.
C. If associated with malignant tumor, as described under the criteria in 13.00 ff.
D. If associated with generalized connective tissue disease, as described under the
criteria in 14.02, 14.03, 14.04, or 14.06.
14.06 Undifferentiated connective tissue disorder. Documented as described in 14.00B5, and with impairment as described under the criteria
in 14.02A, 14.02B, or 14.04.
14.07 Immunoglobulin deficiency syndromes or deficiencies of cell-mediated immunity, excepting
HIV infection. Associated with documented, recurrent severe infection occurring 3 or more times
within a 5-month period.
14.08 Human immunodeficiency virus (HIV) infection. With documentation as described in 14.00D3 and one of the following:
A. Bacterial infections:
1. Mycobacterial infection (e.g., caused by M. avium-intracellulare, M. kansasii,
or M. tuberculosis) at a site other than the lungs, skin, or cervical or hilar lymph
nodes; or pulmonary tuberculosis resistant to treatment; or
2. Nocardiosis; or
3. Salmonella bacteremia, recurrent non-typhoid; or
4. Syphilis or neurosyphilis—evaluate sequelae under the criteria for the affected
body system (e.g., 2.00 Special Senses and Speech, 4.00 Cardiovascular System, 11.00
5. Multiple or recurrent bacterial infection(s), including pelvic inflammatory disease,
requiring hospitalization or intravenous antibiotic treatment 3 or more times in 1
B. Fungal infections:
1. Aspergillosis; or
2. Candidiasis, at a site other than the skin, urinary tract, intestinal tract, or
oral or vulvovaginal mucous membranes; or candidiasis involving the esophagus, trachea,
bronchi, or lungs; or
3. Coccidioidomycosis, at a site other than the lungs or lymph nodes; or
4. Cryptococcosis, at a site other than the lungs (e.g., cryptococcal meningitis);
5. Histoplasmosis, at a site other than the lungs or lymph nodes; or
C. Protozoan or helminthic infections:
1. Cryptosporidiosis, isosporiasis, or microsporidiosis, with diarrhea lasting for
1 month or longer; or
2. Pneumocystis carinii pneumonia or extrapulmonary pneumocystis carinii infection;
3. Strongyloidiasis, extra-intestinal; or
4. Toxoplasmosis of an organ other than the liver, spleen, or lymph nodes.
D. Viral infections:
1. Cytomegalovirus disease (documented as described in 14.00D4b) at a site other than
the liver, spleen, or lymph nodes; or
2. Herpes simplex virus causing:
a. Mucocutaneous infection (e.g., oral, genital, perianal) lasting for 1 month or
b. Infection at a site other than the skin or mucous membranes (e.g., bronchitis,
pneumonitis, esophagitis, or encephalitis); or
c. Disseminated infection; or
3. Herpes zoster, either disseminated or with multidermatomal eruptions that are resistant
to treatment; or
4. Progressive multifocal leukoencephalopathy; or
5. Hepatitis, as described under the criteria in 5.05.
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
c. Involvement of the skin or mucous membranes, as described under the criteria in
3. Lymphoma (e.g., primary lymphoma of the brain, Burkitt's lymphoma, immunoblastic
sarcoma, other non-Hodgkins lymphoma, Hodgkin's disease); or
4. Squamous cell carcinoma of the anus.
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
(e.g., dermatological conditions such as eczema or psoriasis, vulvovaginal or other
mucosal candida, condyloma caused by human papillomavirus, genital ulcerative disease),
or evaluate under the criteria in 8.00 ff.
G. Hematologic abnormalities:
1. Anemia, as described under the criteria in 7.02; or
2. Granulocytopenia, as described under the criteria in 7.15; or
3. Thrombocytopenia, as described under the criteria in 7.06.
H. Neurological abnormalities:
1. HIV encephalopathy, characterized by cognitive or motor dysfunction that limits
function and progresses; or
2. Other neurological manifestations of HIV infection (e.g., peripheral neuropathy)
as described under the criteria in 11.00 ff.
I. HIV wasting syndrome, characterized by involuntary weight loss of 10 percent or
more of baseline (or other significant involuntary weight loss, as described in 14.00D2)
and, in the absence of a concurrent illness that could explain the findings, either:
1. Chronic diarrhea with two or more loose stools daily lasting for 1 month or longer;
2. Chronic weakness and documented fever greater than 38° C (100.4° F) for the majority
of 1 month or longer.
J. Diarrhea, lasting for 1 month or longer, resistant to treatment, and requiring
intravenous hydration, intravenous alimentation, or tube feeding.
K. Cardiomyopathy, as described under the criteria in 4.00ff or 11.04.
L. Nephropathy, as described under the criteria in 6.00 ff.
M. One or more of the following infections (other than described in A-L, above), resistant
to treatment or requiring hospitalization or intravenous treatment 3 or more times
in 1 year (or evaluate sequelae under the criteria for the affected body system).
1. Sepsis; or
2. Meningitis; or
3. Pneumonia; or
4. Septic arthritis; or
5. Endocarditis; or
6. Sinusitis documented by appropriate medically acceptable imaging.
N. Repeated (as defined in 14.00D8) manifestations of HIV infection (including those
listed in 14.08A-M, but without the requisite findings, e.g., carcinoma of the cervix
not meeting the criteria in 14.08E, diarrhea not meeting the criteria in 14.08J, or
other manifestations, e.g., oral hairy leukoplakia, myositis) resulting in significant,
documented, symptoms or signs (e.g., fatigue, fever, malaise, weight loss, pain, night
sweats) and one of the following at the marked level (as defined in 14.00D8) . (See
DI 24595.010 .)
1. Restriction of activities of daily living; or
2. Difficulties in maintaining social functioning; or
3. Difficulties in completing tasks in a timely manner due to deficiencies in concentration,
persistence, or pace. (See DI24595.005 .)
14.09 Inflammatory arthritis. Documented as described in 14.00B6, with one of the following:
A. History of joint pain, swelling, and tenderness, and signs on current physical
examination of joint inflammation or deformity in two or more major joints resulting
in inability to ambulate effectively or inability to perform fine and gross movements
effectively, as defined in 14.00B6b and 1.00B2b and B2c;
B. Ankylosing spondylitis or other spondyloarthropathy, with diagnosis established
by findings of unilateral or bilateral sacroiliitis (e.g., erosions or fusions), shown
by appropriate medically acceptable imaging, with both:
1. History of back pain, tenderness, and stiffness, and
2. Findings on physical examination of ankylosis (fixation) of the dorsolumbar or
cervical spine at 45o or more of flexion measured from the vertical position (zero degrees);
C. An impairment as described under the criteria in 14.02A.
D. Inflammatory arthritis, with signs of peripheral joint inflammation on current
examination, but with lesser joint involvement than in A and lesser extra-articular
features than in C, and:
1. Significant, documented constitutional symptoms and signs (e.g., fatigue, fever,
malaise, weight loss), and
2. Involvement of two or more organs/body systems (see 14.00B6d). At least one of
the organs/body systems must be involved to at least a moderate level of severity.
E. Inflammatory spondylitis or other inflammatory spondyloarthropathies, with lesser
deformity than in B and lesser extra-articular features than in C, with signs of unilateral
or bilateral sacroiliitis on appropriate medically acceptable imaging; and with the
extra-articular features described in 14.09D.