TN 4 (04-21)
DI 34234.017 Immune Listings from 03/27/17 to 04/01/21
114.00
Immune System (Effective Date 01/17/2017)
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 phagocytosis,
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. In children, immune system disorders or their treatment
may also affect growth, development, and the performance of age-appropriate activities.
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 (114.00D). 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 children differ from the features of the
same disorders in adults. The impact of the disorders or their treatment on physical,
psychological, and developmental growth of pre-pubertal children may be considerable,
and often differs from that of post-pubertal adolescents or adults.
3. Immune deficiency disorders, excluding HIV infection (114.00E). 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. Children with immune deficiency disorders also have an increased
risk of malignancies and of having autoimmune disorders.
4. Human immunodeficiency virus (HIV) infection (114.00F). HIV infection may be characterized by increased susceptibility to common infections
as well as opportunistic infections, cancers, or other conditions listed in 114.11.
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.
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 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.
2. 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 101.00B2b.
7. Inability to perform fine and gross movements effectively has the same meaning as in 101.00B2c.
8. Major peripheral joints has the same meaning as in 101.00F.
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.
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 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.
12. 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 process in §416.924.
D. How do we document and evaluate the listed autoimmune disorders?
1. Systemic lupus erythematosus (114.02).
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 auto-antibodies and pro- and
anti-coagulant proteins that may occur in a highly variable pattern.
b. Documentation 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 (114.03).
a. General.
(i) 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) Children can develop the vasculitis of Kawasaki disease, of which the most serious
manifestation is formation of coronary artery aneurysms and related complications.
We evaluate heart problems related to Kawasaki disease under the criteria in the cardiovascular
listings (104.00). Children can also develop the vasculitis of anaphylactoid purpura
(Henoch-Schoenlein purpura), which may cause intestinal and renal disorders. We evaluate
intestinal and renal disorders related to vasculitis of anaphylactoid purpura under
the criteria in the digestive (105.00) or genitourinary (106.00) listings. Other clinical
patterns include, but are not limited to, polyarteritis nodosa, Takayasu's arteritis
(aortic arch arteritis), and Wegener's granulomatosis.
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) (114.04).
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).
(i) Localized scleroderma (linear scleroderma and morphea) is more common in children
than systemic scleroderma. 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 (101.00).
(ii) 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 (102.00) or mental disorders (112.00).
(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 (101.00) or respiratory system listings (103.00).
d. Documentation of systemic sclerosis (scleroderma). Documentation involves differentiating the clinical features of systemic sclerosis
(scleroderma) from other autoimmune disorders. However, there may be an overlap .
4. Polymyositis and dermatomyositis (114.05).
a. General.
(i) 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. 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.
(ii) Polymyositis occurs rarely in children; the more common presentation in children
is dermatomyositis with symmetric proximal muscle weakness and characteristic skin
findings. The clinical course of dermatomyositis can be more severe when it is accompanied
by systemic vasculitis rather than just localized to striated muscle. Late in the
disease, some children with dermatomyositis develop calcinosis of the skin and subcutaneous
tissues, muscles, and joints. We evaluate the involvement of other organs/body systems
under the criteria for the listings in the affected body system.
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 children, the diagnosis of
dermatomyositis is supported largely by medical history, findings on physical examination
that include the characteristic skin findings, and elevated serum muscle enzymes.
Muscle inflammation or vasculitis depicted on MRI is additional evidence supporting
the diagnosis of childhood dermatomyositis. When you have had electromyography, muscle
biopsy, or MRI 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, muscle biopsy, or MRI.
c. Additional information about how we evaluate polymyositis and dermatomyositis
under the listings.
(i) In newborn and younger infants (birth to attainment of age 1), we consider muscle
weakness that affects motor skills, such as head control, reaching, grasping, taking
solids, or self-feeding, under 114.05A. In older infants and toddlers (age 1 to attainment
of age 3), we also consider muscle weakness affecting your ability to roll over, sit,
crawl, or walk under 114.05A.
(ii) If you are of preschool age through adolescence (age 3 to attainment of age 18),
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 under 114.05A.
5. Undifferentiated and mixed connective tissue disease (114.06).
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. The most
common pattern of undifferentiated autoimmune disorders in children is mixed connective
tissue disease (MCTD).
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. Children with MCTD have laboratory findings of extremely high
antibody titers to extractable nuclear antigen (ENA) or ribonucleoprotein (RNP) without
high titers of anti-dsDNA or anti-SM antibodies. There are often clinical findings
suggestive of SLE or childhood dermatomyositis. Many children later develop features
of scleroderma.
6. Inflammatory arthritis (114.09).
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. You may
also have impaired growth as a result of the inflammatory arthritis because of its
effects on the immature skeleton, open epiphyses, and young cartilage and bone. We
evaluate any associated growth impairment under the criteria in 100.00.
b. Inflammatory arthritis involving the axial spine (spondyloarthropathy). In children, inflammatory arthritis involving the axial spine may be associated with
disorders such as:
(i) Reactive arthropathies;
(ii) Juvenile ankylosing spondylitis;
(iii) Psoriatic arthritis;
(iv) SEA syndrome (seronegative enthesopathy arthropathy syndrome);
(v) Behçet's disease; and
(vi) Inflammatory bowel disease.
c. Inflammatory arthritis involving the peripheral joints. In children, inflammatory arthritis involving peripheral joints may be associated
with disorders such as:
(i) Juvenile rheumatoid arthritis;
(ii) Sjögren's syndrome;
(iii) Psoriatic arthritis;
(iv) Crystal deposition disorders (gout and pseudogout);
(v) Lyme disease; and
(vi) Inflammatory bowel disease.
d. Documentation of inflammatory arthritis. Generally, but not always, the diagnosis of inflammatory arthritis is based on the
clinical features and serologic findings described in the most recent edition of the
"Primer on the Rheumatic Diseases" published by the Arthritis Foundation.
e. How we evaluate inflammatory arthritis under the listings.
(i) Listing-level severity in 114.09A and 114.09C1 is shown by an impairment that
results in an "extreme" (very serious) limitation. In 114.09A, the criterion is satisfied
with persistent inflammation or deformity in one major peripheral weight-bearing joint
resulting in the inability to ambulate effectively (as defined in 114.00C6) or one
major peripheral joint in each upper extremity resulting in the inability to perform
fine and gross movements effectively (as defined in 114.00C7). In 114.09C1, if you
have the required ankylosis (fixation) of your cervical or dorsolumbar spine, we will
find that you have an extreme limitation in your ability to see in front of you, above
you, and to the side. Therefore, inability to ambulate effectively is implicit in
114.09C1, even though you might not require bilateral upper limb assistance.
(ii) Listing-level severity is shown in 114.09B and 114.09C2 by inflammatory arthritis
that involves various combinations of complications of one or more major peripheral
joints or involves other joints, such as inflammation or deformity, extra-articular
features, repeated manifestations, and constitutional symptoms and signs. Extra-articular
impairments may also meet listings in other body systems.
(iii) Extra-articular features of inflammatory arthritis may involve any body system;
for example: Musculoskeletal (heel enthesopathy), ophthalmologic (iridocyclitis, keratoconjunctivitis
sicca, uveitis), pulmonary (pleuritis, pulmonary fibrosis or nodules, restrictive
lung disease), cardiovascular (aortic valve insufficiency, arrhythmias, coronary arteritis,
myocarditis, pericarditis, Raynaud's phenomenon, systemic vasculitis), renal (amyloidosis
of the kidney), hematologic (chronic anemia, thrombocytopenia), neurologic (peripheral
neuropathy, radiculopathy, spinal cord or cauda 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 (114.10).
a. General.
(i) Sjögren's syndrome is an immune-mediated 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 disorder predominate.
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?
1. General.
a. Immune deficiency disorders can be classified as:
(i) Primary (congenital); for example, X-linked 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.
3. Immune 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 infections.
(iii) Significant deterioration of other organ systems.
4. 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.
c. Significant deterioration of other organ systems.
F. How do we document and evaluate HIV infection?
Any child with HIV infection, including one with a diagnosis of acquired immune deficiency
syndrome (AIDS), may be found disabled under 114.11 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.
a. Definitive documentation of HIV infection. We may document a diagnosis of HIV infection by positive findings on one or more of
the following definitive laboratory tests:
(i) HIV antibody screening test (for example, enzyme immunoassay, or EIA), confirmed
by a supplemental HIV antibody test such as the Western blot (immunoblot) or immunofluorescence
assay, for any child age 18 months or older.
(ii) HIV nucleic acid (DNA or RNA) detection test (for example, polymerase chain reaction,
or PCR).
(iii) HIV p24 antigen (p24Ag) test, for any child age 1 month or older.
(iv) Isolation of HIV in viral culture.
(v) Other tests that are highly specific for detection of HIV and that are consistent
with the prevailing state of medical knowledge.
b. We will make every reasonable effort to obtain the results of your laboratory testing.
Pursuant to § 416.919f, we will purchase examinations or tests necessary to make a
determination in your claim if no other acceptable documentation exists.
c. Other acceptable documentation of HIV infection. We may also document HIV infection without definitive laboratory evidence.
(i) We will accept a persuasive report from a physician that a positive diagnosis
of your HIV infection was confirmed by an appropriate laboratory test(s), such as
those described in 114.00F1a. To be persuasive, this report must state that you had
the appropriate definitive laboratory test(s) for diagnosing your HIV infection and
provide the results. The report must also be consistent with the remaining evidence
of record.
(ii) We may also document HIV infection by the medical history, clinical and laboratory
findings, and diagnosis(es) indicated in the medical evidence, 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,
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 or Pneumocystis
pneumonia (PCP)), and there is no other known cause of diminished resistance to that
disease (for example, long-term steroid treatment or lymphoma). In such cases, we
will make every reasonable effort to obtain full details of the history, medical findings,
and results of testing.
2. Documentation of the manifestations of HIV infection.
a. Definitive documentation of manifestations of HIV infection. We may document manifestations of HIV infection by positive findings on definitive
laboratory tests, such as culture, microscopic examination of biopsied tissue or other
material (for example, bronchial washings), serologic tests, or on other generally
acceptable definitive tests consistent with the prevailing state of medical knowledge
and clinical practice.
b. We will make every reasonable effort to obtain the results of your laboratory testing.
Pursuant to § 416.919f, we will purchase examinations or tests necessary to make a
determination of your claim if no other acceptable documentation exists.
c. Other acceptable documentation of manifestations of HIV infection. We may also document manifestations of HIV infection without definitive laboratory
evidence.
(i) We will accept a persuasive report from a physician that a positive diagnosis
of your manifestation of HIV infection was confirmed by an appropriate laboratory
test(s). To be persuasive, this report must state that you had the appropriate definitive
laboratory test(s) for diagnosing your manifestation of HIV infection and provide
the results. The report must also be consistent with the remaining evidence of record.
(ii) We may also document manifestations of HIV infection without the definitive laboratory
evidence described in 114.00F2a, 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.
3. Disorders associated with HIV infection (114.11A-E).
a. Multicentric Castleman disease. (MCD, 114.11A) affects multiple groups of lymph nodes and organs containing lymphoid
tissue. This widespread involvement distinguishes MCD from localized (or unicentric)
Castleman disease, which affects only a single set of lymph nodes. While not a cancer,
MCD is known as a lymphoproliferative disorder. Its clinical presentation and progression
is similar to that of lymphoma, and its treatment may include radiation or chemotherapy.
We require characteristic findings on microscopic examination of the biopsied lymph
nodes or other generally acceptable methods consistent with the prevailing state of
medical knowledge and clinical practice to establish the diagnosis. Localized (or
unicentric) Castleman disease does not meet or medically equal the criterion in 114.11A,
but we may evaluate it under the criteria in 114.11G or 14.11I in part A.
b. Primary central nervous system lymphoma. (PCNSL, 114.11B) originates in the brain, spinal cord, meninges, or eye. Imaging tests
(for example, MRI) of the brain, while not diagnostic, may show a single lesion or
multiple lesions in the white matter of the brain. We require characteristic findings
on microscopic examination of the cerebral spinal fluid or of the biopsied brain tissue,
or other generally acceptable methods consistent with the prevailing state of medical
knowledge and clinical practice to establish the diagnosis.
c. Primary effusion lymphoma (PEL, 114.11C) is also known as body cavity lymphoma. We require characteristic findings
on microscopic examination of the effusion fluid or of the biopsied tissue from the
affected internal organ, or other generally acceptable methods consistent with the
prevailing state of medical knowledge and clinical practice to establish the diagnosis.
d. Progressive multifocal leukoencephalopathy (PML, 114.11D) is a progressive neurological degenerative syndrome caused by the
John Cunningham (JC) virus in immunosuppressed children. Clinical findings of PML
include clumsiness, progressive weakness, and visual and speech changes. Personality
and cognitive changes may also occur. We require appropriate clinical findings, characteristic
white matter lesions on MRI, and a positive PCR test for the JC virus in the cerebrospinal
fluid to establish the diagnosis. We also accept a positive brain biopsy for JC virus
or other generally acceptable methods consistent with the prevailing state of medical
knowledge and clinical practice to establish the diagnosis.
e. Pulmonary Kaposi sarcoma (Kaposi sarcoma in the lung, 114.11E) is the most serious form of Kaposi sarcoma (KS).
Other internal KS tumors (for example, tumors of the gastrointestinal tract) have
a more variable prognosis. We require characteristic findings on microscopic examination
of the induced sputum, bronchoalveolar lavage washings, or of the biopsied transbronchial
tissue, or other generally acceptable methods consistent with the prevailing state
of medical knowledge and clinical practice to establish the diagnosis.
4. CD4 measurement (114.11F). To evaluate your HIV infection under 114.11F, we require one measurement of your absolute
CD4 count (also known as CD4 count or CD4+ T-helper lymphocyte count) or CD4 percentage
for children from birth to attainment of age 5, or one measurement of your absolute
CD4 count for children from age 5 to attainment of age 18. These measurements (absolute
CD4 count or CD4 percentage) must occur within the period we are considering in connection
with your application or continuing disability review. If you have more than one CD4
measurement within this period, we will use your lowest absolute CD4 count or your
lowest CD4 percentage.
5. Complications of HIV infection requiring hospitalization
(114.11G).
a. Complications of HIV infection may include infections (common or opportunistic),
cancers, and other conditions. Examples of complications that may result in hospitalization
include: Depression; diarrhea; immune reconstitution inflammatory syndrome; malnutrition;
and PCP and other severe infections.
b. Under 114.11G, we require three hospitalizations within a 12-month period that
are at least 30 days apart and that result from a complication(s) of HIV infection.
The hospitalizations may be for the same complication or different complications of
HIV infection and are not limited to the examples of complications that may result
in hospitalization listed in 114.00F5a. All three hospitalizations must occur within
the period we are considering in connection with your application or continuing disability
review. Each hospitalization must last at least 48 hours, including hours in a hospital
emergency department immediately before the hospitalization.
c. We will use the rules on medical equivalence in § 416.926 to evaluate your HIV
infection if you have fewer, but longer, hospitalizations, or more frequent, but shorter,
hospitalizations, or if you receive nursing, rehabilitation, or other care in alternative
settings.
6. Neurological manifestations specific to children (114.11H). The methods of identifying
and evaluating neurological manifestations 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 CT scan or MRI. Neurological manifestations may present in the
loss of acquired developmental milestones (developmental regression) in infants and
young children or, in the loss of acquired intellectual abilities in school-age children
and adolescents. A child may demonstrate loss of intellectual abilities by a decrease
in IQ scores, by forgetting information previously learned, by inability to learn
new information, or by a sudden onset of a new learning disability. When infants and
young children present with serious developmental delays (without regression), we
evaluate the child's impairment(s) under 112.00.
7. Growth failure due to HIV immune suppression (114.11I).
a. To evaluate growth failure due to HIV immune suppression, we require documentation
of the laboratory values described in 114.11I1 and the growth measurements in 114.11I2
or 114.11I3 within the same consecutive 12-month period. The dates of laboratory findings
may be different from the dates of growth measurements.
b. Under 114.11I2 and 114.11I3, we use the appropriate table under 105.08B in the
digestive system to determine whether a child's growth is less than the third percentile.
(i) For children from birth to attainment of age 2, we use the weight-for-length table
corresponding to the child's gender (Table I or Table II).
(ii) For children from age 2 to attainment of age 18, we use the body mass index (BMI)-for-age
corresponding to the child's gender (Table III or Table IV).
(iii) BMI is the ratio of a child's weight to the square of his or her height. We
calculate BMI using the formulas in 105.00G2c.
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.
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 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, 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 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 114.00G1 and 114.00G2. Long-term corticosteroid
treatment can cause ischemic necrosis of bone, posterior subcapsular cataract, impaired
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 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 114.00G1 and 114.00G2. A frequent
need for treatment such as intravenous immunoglobulin 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,
shortness of breath, or limitations in mental function including cognition (for example,
memory) concentration, and mood.
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 114.00G1
and 114.00G2. 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 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, 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 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 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 §§ 416.921 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.
NOTE: SSR 96-7p, "Titles II and XVI: Evaluation of Symptoms in Disability Claims: Assessing
the Credibility of an Individual's Statements" has been replaced with SSR 16-3p, "Titles
II and XVI: Evaluation of Symptoms in Disability Claims." Effective 3/28/16, we no
longer use the term "credibility" when evaluating symptoms.
I. How do we consider the impact of your immune system disorder on your
functioning?
1. We will consider all relevant information in your case record to determine the
full impact of your immune system disorder, including HIV infection, on your ability
to function. 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,
diarrhea, severe fatigue, or pain, resulting in a limitation of your ability to acquire
information, to concentrate, to persevere at a task, to interact with others, to move
about, or to cope with stress. You may also have limitations because of your treatment
and its side effects (see 114.00G).
2. Important factors we will consider when we evaluate your functioning include, but
are not limited to: Your symptoms (see 114.00H), 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 (see 114.00G).
See §§416.924a and 416.926a for additional guidance on the factors we consider when
we evaluate your functioning.
3. We will use the rules in §§ 416.924a and 416.926a to evaluate your functional limitations
and determine whether your impairment functionally equals the listings
J. How do we evaluate your immune system disorder when it does not meet one of the
listings?
1. These listings are only examples of immune system disorders that we consider severe
enough to result in marked and severe functional limitations. 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. Children with immune system disorders, including HIV infection, may manifest signs
or symptoms of a mental impairment or of another physical impairment. For example,
HIV infection may accelerate the onset of conditions such as diabetes or affect the
course of or treatment options for diseases such as cardiovascular disease or hepatitis.
We may evaluate these impairments under the affected body system.
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 Cardiovascular 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) medically 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.
114.01 Category of Impairments, Immune System Disorders
114.02
Systemic
lupus
erythematosus, as described in 114.00D1. With involvement of two or more organs/body systems, and
with:
A. One of the organs/body systems involved to at least a moderate level of severity;
AND
B. At least two of the constitutional symptoms and signs (severe fatigue, fever, malaise,
or involuntary weight loss).
114.03
Systemic
vasculitis, as described in 114.00D2. With involvement of two or more organs/body systems, and
with:
A. One of the organs/body systems involved to at least a moderate level of severity;
AND
B. At least two of the constitutional symptoms and signs (severe fatigue, fever, malaise,
or involuntary weight loss).
114.04
Systemic
sclerosis
(scleroderma). As described in 114.00D3. With:
A. Involvement of two or more organs/body systems, with:
1. One of the organs/body systems involved to at least a moderate level of severity;
and
2. At least two of the constitutional symptoms or signs (severe fatigue, fever, malaise,
or involuntary weight loss).
OR
B. With one of the following:
1. Toe contractures or fixed deformity of one or both feet, resulting in the inability
to ambulate effectively as defined in 114.00C6; or
2. Finger contractures or fixed deformity in both hands, resulting in the inability
to perform fine and gross movements effectively as defined in 114.00C7; or
3. Atrophy with irreversible damage in one or both lower extremities, resulting in
the inability to ambulate effectively as defined in 114.00C6; or
4. Atrophy with irreversible damage in both upper extremities, resulting in the inability
to perform fine and gross movements effectively as defined in 114.00C7.
OR
C. Raynaud's phenomenon, characterized by:
1. Gangrene involving at least two extremities; or
2. Ischemia with ulcerations of toes or fingers, resulting in the inability to ambulate
effectively or to perform fine and gross movements effectively as defined in 114.00C6
and 114.00C7.
114.05
Polymyositis
and
dermatomyositis. As described in 114.00D4. With:
A. Proximal limb-girdle (pelvic or shoulder) muscle weakness, resulting in inability
to ambulate effectively or inability to perform fine and gross movements effectively
as defined in 114.00C6 and 114.00C7.
OR
B. Impaired swallowing (dysphagia) with aspiration due to muscle weakness.
OR
C. Impaired respiration due to intercostal and diaphragmatic muscle weakness.
OR
D. Diffuse calcinosis with limitation of joint mobility or intestinal motility.
114.06
Undifferentiated
and mixed connective tissue
disease, as described in 114.00D5. With involvement of two or more organs/body systems, and
with:
A. One of the organs/body systems involved to at least a moderate level of severity;
AND
B. At least two of the constitutional symptoms and signs (severe fatigue, fever, malaise,
or involuntary weight loss).
114.07
Immune
deficiency disorders, excluding HIV
infection. As described in 114.00E. With:
A. One or more of the following infections. 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
B. Stem cell transplantation as described under 114.00E3. Consider under a disability
until at least 12 months from the date of transplantation. Thereafter, evaluate any
residual impairment(s) under the criteria for the affected body system.
114.08
[Reserved]
114.09
Inflammatory
arthritis. As described in 114.00D6. With:
A. Persistent inflammation or persistent deformity of:
1. One or more major peripheral weight-bearing joints resulting in the inability to
ambulate effectively (as defined in 114.00C6); or
2. One or more major peripheral joints in each upper extremity resulting in the inability
to perform fine and gross movements effectively (as defined in 114.00C7).
OR
B. Inflammation or deformity in one or more major peripheral joints with:
1. Involvement of two or more organs/body systems with one of the organs/body systems
involved to at least a moderate level of severity; and
2. At least two of the constitutional symptoms or signs (severe fatigue, fever, malaise,
or involuntary weight loss).
OR
C. Ankylosing spondylitis or other spondyloarthropathies, with:
1. Ankylosis (fixation) of the dorsolumbar or cervical spine as shown by appropriate
medically acceptable imaging and measured on physical examination at 45º or more of
flexion from the vertical position (zero degrees); or
2. Ankylosis (fixation) of the dorsolumbar or cervical spine as shown by appropriate
medically acceptable imaging and measured on physical examination at 30 or more of
flexion (but less than 45) measured from the vertical position (zero degrees), and
involvement of two or more organs/body systems with one of the organs/body systems
involved to at least a moderate level of severity.
114.10
Sjögren's
syndrome, as described in 114.00D7. With involvement of two or more organs/body systems, and
with:
A. One of the organs/body systems involved to at least a moderate level of severity;
AND
B. At least two of the constitutional symptoms and signs (severe fatigue, fever, malaise,
or involuntary weight loss).
114.11
Human
immunodeficiency virus (HIV)
infection. With documentation as described in 114.00F1 and one of the following:
A. Multicentric (not localized or unicentric) Castleman disease affecting multiple
groups of lymph nodes or organs containing lymphoid tissue (see 114.00F3a).
OR
B. Primary central nervous system lymphoma (see 114.00F3b).
OR
C. Primary effusion lymphoma (see 114.00F3c).
OR
D. Progressive multifocal leukoencephalopathy (see 114.00F3d).
OR
E. Pulmonary Kaposi sarcoma (see 114.00F3e).
OR
F. Absolute CD4 count or CD4 percentage (see 114.00F4):
1. For children from birth to attainment of age 1, absolute CD4 count of 500 cells/mm3
or less, or CD4 percentage of less than 15 percent; or
2. For children from age 1 to attainment of age 5, absolute CD4 count of 200 cells/mm3
or less, or CD4 percentage of less than 15 percent; or
3. For children from age 5 to attainment of age 18, absolute CD4 count of 50 cells/mm3
or less.
OR
G. Complication(s) of HIV infection requiring at least three hospitalizations within
a 12-month period and at least 30 days apart (see 114.00F5). Each hospitalization
must last at least 48 hours, including hours in a hospital emergency department immediately
before the hospitalization.
OR
H. A neurological manifestation of HIV infection (for example, HIV encephalopathy or
peripheral neuropathy) (see 114.00F6) resulting in one of the following:
1. Loss of previously acquired developmental milestones or intellectual ability (including
the sudden onset of a new learning disability), documented on two examinations at
least 60 days apart; or
2. Progressive motor dysfunction affecting gait and station or fine and gross motor
skills, documented on two examinations at least 60 days apart; or
3. Microcephaly with head circumference that is less than the third percentile for
age, documented on two examinations at least 60 days apart; or
4. Brain atrophy, documented by appropriate medically acceptable imaging.
OR
I. Immune suppression and growth failure (see 114.00F7) documented by 1 and 2, or by
1 and 3:
1. CD4 measurement:
a. For children from birth to attainment of age 5, CD4 percentage of less than 20
percent; or
b. For children from age 5 to attainment of age 18, absolute CD4 count of less than
200 cells/mm3 or CD4 percentage of less than 14 percent; and
2. For children from birth to attainment of age 2, three weight-for-length measurements
that are:
a. Within a consecutive 12-month period; and
b. At least 60 days apart; and
c. Less than the third percentile on the appropriate weight-for-length table under
105.08B1; or
3. For children from age 2 to attainment of age 18, three BMI-for-age measurements
that are:
a. Within a consecutive 12-month period; and
b. At least 60 days apart; and
c. Less than the third percentile on the appropriate BMI-for-age table under 105.08B2.