TN 2 (01-17)
DI 34234.013 Immune Listings from 06/12/15 to 01/16/17
114.00 Immune System (Effective Date 06/12/2015)
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 opportunistic infections,
cancers, or other conditions, as described in 114.08.
B. What information do we need to show that you have an immune system disorder?
Generally, we need your medical history, a report(s) of a physical examination, a
report(s) of laboratory findings, and in some instances, appropriate medically acceptable
imaging or tissue biopsy reports to show that you have an immune system disorder.
Therefore, we will make every reasonable effort to obtain your medical history, medical
findings, and results of laboratory tests. We explain the information we need in more
detail in the sections below.
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 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, 114.09C2, and 114.09D 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 human immunodeficiency virus (HIV) infection?
Any child with HIV infection, including one with a diagnosis of acquired immune deficiency
syndrome (AIDS), may be found disabled under 114.08 if his or her impairment meets
the criteria in that listing or is medically equivalent to the criteria in that listing.
1. Documentation of HIV infection. The medical evidence must include documentation of HIV infection. Documentation may
be by laboratory evidence or by other generally acceptable methods consistent with
the prevailing state of medical knowledge and clinical practice. When you have had
laboratory testing for HIV infection, we will make every reasonable effort to obtain
reports of the results of that testing. However, we will not purchase laboratory testing
to establish whether you have HIV infection.
a. Definitive documentation of HIV infection. A definitive diagnosis of HIV infection is documented by one or more of the following
laboratory tests:
(i) HIV antibody tests. HIV antibodies are usually first detected by an ELISA screening
test performed on serum. Because the ELISA can yield false positive results, confirmation
is required using a more definitive test, such as a Western blot or an immunofluorescence
assay. Positive results on these tests are considered to be diagnostic of HIV infection
in a child age 18 months or older. (See b. below for information about HIV antibody
testing in children younger than 18 months of age.)
(ii) Positive “viral load” (VL) tests. These tests are normally used to quantitate
the amount of the virus present but also document HIV infection. Such tests include
the quantitative plasma HIV RNA, quantitative plasma HIV branched DNA, and reverse
transcriptase-polymerase chain reaction (RT-PCR).
(iii) HIV DNA detection by polymerase chain reaction (PCR).
(iv) A specimen that contains HIV antigen (for example, serum specimen, lymphocyte
culture, or cerebrospinal fluid) in a child age 1 month or older.
(v) A positive viral culture for HIV from peripheral blood mononuclear cells (PBMC).
(vi) An immunoglobulin A (IgA) serological assay that is specific for HIV.
(vii) Other tests that are highly specific for detection of HIV and that are consistent
with the prevailing state of medical knowledge.
b. Definitive documentation of HIV infection in children from birth to the attainment
of 18 months. For children from birth to the attainment of 18 months of age, and who have tested
positive for HIV antibodies, HIV infection is documented by:
(i) One or more of the tests listed in F1a(ii)-F1a(vii).
(ii) For newborn and younger infants (birth to attainment of age 1), a CD4 (T4) count
of 1500/mm3 or less, or a CD4 count less than or equal to 20 percent of total lymphocytes.
(iii) For older infants and toddlers from 12 to 18 months of age, a CD4 (T4) count
of 750/mm3 or less, or a CD4 count less than or equal to 20 percent of total lymphocytes.
(iv) An abnormal CD4/CD8 ratio.
(v) A severely diminished immunoglobulin G (IgG) level (< 4 g/l or 400 mg/dl), or
significantly greater than normal range for age.
c. Other acceptable documentation of HIV infection. We may also document HIV infection without the definitive laboratory evidence described
in 114.00F1a, provided that such documentation is consistent with the prevailing state
of medical knowledge and clinical practice and is consistent with the other evidence
in your case record. If no definitive laboratory evidence is available, we may document
HIV infection by the medical history, clinical and laboratory findings, and diagnosis(es)
indicated in the medical evidence. For example, we will accept a diagnosis of HIV
infection without definitive laboratory evidence of the HIV infection if you have
an opportunistic disease that is predictive of a defect in cell-mediated immunity
(for example, Pneumocystis pneumonia (PCP)), and there is no other known cause of diminished resistance to that
disease (for example, long-term steroid treatment, lymphoma). In such cases, we will
make every reasonable effort to obtain full details of the history, medical findings,
and results of testing.
2. CD4 tests. Children who have HIV infection or other disorders of the immune system may have
tests showing a reduction of either the absolute count or the percentage of their
T-helper lymphocytes (CD4 cells). The extent of immune suppression correlates with
the level or rate of decline of the CD4 count (relative to the age of the young child).
By age 6, children have CD4 counts comparable to those levels found in adults. Generally,
in these children when the CD4 count is below 200/mm3 (or below 14 percent of the total lymphocyte count) the susceptibility to opportunistic
infection is greatly increased. Although a reduced CD4 count alone does not establish
a definitive diagnosis of HIV infection, a CD4 count below 200 does offer supportive
evidence when there are clinical findings, but not a definitive diagnosis of an opportunistic
infection(s). However, a reduced CD4 count alone does not document the severity or functional consequences of HIV infection.
3. Documentation of the manifestations of HIV infection. The medical evidence must also include documentation of the manifestations of HIV
infection. Documentation may be by laboratory evidence or other generally acceptable
methods consistent with the prevailing state of medical knowledge and clinical practice.
a. Definitive documentation of the manifestations of HIV infection. The definitive method of diagnosing opportunistic diseases or conditions that are
manifestations of HIV infection is by culture, serologic test, or microscopic examination
of biopsied tissue or other material (for example, bronchial washings). We will make
every reasonable effort to obtain specific laboratory evidence of an opportunistic
disease or other condition whenever this information is available. If a histologic
or other test has been performed, the evidence should include a copy of the appropriate
report. If we cannot obtain the report, the summary of hospitalization or a report
from the treating source should include details of the findings and results of the
diagnostic studies (including appropriate medically acceptable imaging studies) or
microscopic examination of the appropriate tissues or body fluids.
b. Other acceptable documentation of the manifestations of HIV infection. We may also document manifestations of HIV infection without the definitive laboratory
evidence described in 114.00F3a, provided that such documentation is consistent with
the prevailing state of medical knowledge and clinical practice and is consistent
with the other evidence in your case record. For example, many conditions are now
commonly diagnosed based on some or all of the following: Medical history, clinical
manifestations, laboratory findings (including appropriate medically acceptable imaging),
and treatment responses. In such cases, we will make every reasonable effort to obtain
full details of the history, medical findings, and results of testing. The following
are examples of how we may document manifestations of HIV infection with other appropriate
evidence.
(i) Although a definitive diagnosis of PCP requires identifying the organism in bronchial
washings, induced sputum, or lung biopsy, these tests are frequently bypassed if PCP
can be diagnosed presumptively. Supportive evidence may include: Fever, dyspnea, hypoxia,
CD4 count below 200 in children 6 years of age or older, and no evidence of bacterial
pneumonia. Also supportive are bilateral lung interstitial infiltrates on x-ray, a
typical pattern on CAT scan, or a gallium scan positive for pulmonary uptake. Response
to anti-PCP therapy usually requires 5-7 days, and such a response can be supportive
of the diagnosis.
(ii) Documentation of Cytomegalovirus (CMV) disease (114.08D) may present special problems because definitive diagnosis
(except for chorioretinitis, which may be diagnosed by an ophthalmologist or optometrist
on funduscopic examination) requires identification of viral inclusion bodies or a
positive culture from the affected organ and the absence of any other infectious agent
likely to be causing the disease. A positive serology test does not establish a definitive
diagnosis of CMV disease, but does offer supportive evidence of a presumptive diagnosis
of CMV disease. Other clinical findings that support a presumptive diagnosis of CMV
may include: Fever, urinary culture positive for CMV, and CD4 count below 200 in children
6 years of age or older. A clear response to anti-CMV therapy also supports a diagnosis.
(iii) A definitive diagnosis of toxoplasmosis of the brain is based on brain biopsy,
but this procedure carries significant risk and is not commonly performed. This condition
is usually diagnosed presumptively based on symptoms or signs of fever, headache,
focal neurologic deficits, seizures, typical lesions on brain imaging, and a positive
serology test.
(iv) Candidiasis of the esophagus (also known as Candida esophagitis) may be presumptively diagnosed based on symptoms of retrosternal pain
on swallowing (odynophagia) and either oropharyngeal thrush (white patches or plaques)
diagnosed on physical examination or by microscopic documentation of Candida fungal elements from a noncultured specimen scraped from the oral mucosa. Treatment
with oral (systemic) antifungal agents usually produces improvement after 5 or more
days of therapy, and such a response can be supportive of the diagnosis.
4. HIV infection manifestations specific to children.
a. General. The clinical manifestation and course of disease in children who become infected
with HIV perinatally or in the first 12 years of life may differ from that in adolescents
(age 12 to attainment of age 18) and adults. Newborn and younger infants (birth to
attainment of age 1) and older infants and toddlers (age 1 to attainment of age 3)
may present with failure to thrive or PCP; preschool children (age 3 to attainment
of age 6) and primary school children (age 6 to attainment of age 12) may present
with recurrent infections, neurological problems, or developmental abnormalities.
Adolescents may also exhibit neurological abnormalities, such as HIV encephalopathy,
or have growth problems. HIV infection that affects the digestive system and results
in malnutrition also may be evaluated under 105.08.
b. Neurologic abnormalities. The methods of identifying and evaluating neurologic abnormalities may vary depending
on a child's age. For example, in an infant, impaired brain growth can be documented
by a decrease in the growth rate of the head. In an older child, impaired brain growth
may be documented by brain atrophy on a CAT scan or MRI. Neurologic abnormalities
in infants and young children may present as serious developmental delays or in the
loss of previously acquired developmental milestones. In school-age children and adolescents,
this type of neurologic abnormality generally presents as the loss of previously acquired
intellectual abilities. This may be evidenced in a child by a decrease in intelligence
quotient (IQ) scores, by forgetting information previously learned, by inability to
learn new information, or by a sudden onset of a new learning disability.
c. Bacterial infections. Children with HIV infection may contract any of a broad range of bacterial infections.
Certain major infections caused by pyogenic bacteria (for example, some pneumonias)
can be severely limiting, especially in pre-adolescent children. We evaluate these
major bacterial infections under 114.08A4. Although 114.08A4 applies only to children
under 13 years of age, children age 13 and older may have an impairment that medically
equals this listing if the circumstances of the case warrant; for example, if there
is delayed puberty. We will evaluate pelvic inflammatory disease in older girls under
114.08A5.
d. Growth failure due to HIV immune suppression.
(i) To evaluate growth failure due to HIV immune suppression, we require documentation
of the laboratory values described in 114.08H1 and the growth measurements in 114.08H2
or 114.08H3 within the same consecutive 12-month period. The dates of laboratory findings
may be different from the dates of growth measurements.
(ii) Under 114.08H2 and 114.08H3, 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.
A. 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).
B. For children 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 IV0.
C. 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 §§416.928, and
416.929. Additionally, when we assess the credibility of your complaints about your
symptoms and their functional effects, we will not draw any inferences from the fact
that you do not receive treatment or that you are not following treatment without
considering all of the relevant evidence in your case record, including any explanations
you provide that may explain why you are not receiving or following treatment.
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 use the functional criteria in these listings?
1. The following listings in this body system include standards for evaluating the
functional limitations resulting from immune system disorders: 114.02B, for systemic
lupus erythematosus; 114.03B, for systemic vasculitis; 114.04D, for systemic sclerosis
(scleroderma); 114.05E, for polymyositis and dermatomyositis; 114.06B, for undifferentiated
and mixed connective tissue disease; 114.07C, for immune deficiency disorders, excluding
HIV infection; 114.08L, for HIV infection; 114.09D, for inflammatory arthritis; and
114.10B, for Sjögren’s syndrome.
2. When we use one of the listings cited in 114.00I1, we will consider all relevant
information in your case record to determine the full impact of your immune system
disorder on your ability to function. Important factors we will consider when we evaluate
your functioning under these listings include, but are not limited to: Your symptoms,
the frequency and duration of manifestations of your immune system disorder, periods
of exacerbation and remission, and the functional impact of your treatment, including
the side effects of your medication.
3. To satisfy the functional criterion in a listing, your immune system disorder must
result in an “extreme” limitation in one domain of functioning or a “marked” limitation
in two domains of functioning depending on your age. (See 112.00C for additional discussion
of these areas of functioning and §§416.924a and 416.926a for additional guidance
on the evaluation of functioning in children.) Functional limitation may result from
the impact of the disease process itself on your mental functioning, physical functioning,
or both your mental and physical functioning. This could result from persistent or
intermittent symptoms, such as depression, severe fatigue, or pain, resulting in a
limitation of your ability to do a task, to concentrate, to persevere at a task, or
to perform the task at an acceptable rate of speed. You may also have limitations
because of your treatment and its side effects (see 114.00G).
J. How do we evaluate your immune system disorder when it does not meet one of these
listings?
1. These listings are only examples of immune system disorders that we consider severe
enough to 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. Individuals with immune system disorders, including HIV infection, may manifest
signs or symptoms of a mental impairment or of another physical impairment. We may
evaluate these impairments under any affected body system. For example, we will evaluate:
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:
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. Any other manifestation(s) of SLE resulting in one of the following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age-group
criteria in paragraph B2 of 112.02.
114.03 Systemic vasculitis As described in 114.00D2. 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. Any other manifestation(s) of systemic vasculitis resulting in one of the following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age-group
criteria in paragraph B2 of 112.02.
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;
OR
D. Any other manifestation(s) of systemic sclerosis (scleroderma) resulting in one of
the following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age-group
criteria in paragraph B2 of 112.02.
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.
OR
E. Any other manifestation(s) of polymyositis or dermatomyositis resulting in one of
the following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age-group
criteria in paragraph B2 of 112.02.
114.06 Undifferentiated and mixed connective tissue disease. As described in 114.00D5. 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. Any other manifestation(s) of undifferentiated or mixed connective tissue disease
resulting in one of the following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age-group
criteria in paragraph B2 of 112.02.
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.
OR
C. Any other manifestation(s) of an immune deficiency disorder resulting in one of the
following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age-group
criteria in paragraph B2 of 112.02.
114.08 Human immunodeficiency virus (HIV) infection. With documentation as described in 114.00F and one of the following:
A. Bacterial infections:
1. Mycobacterial infection (for example, 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. In a child less than 13 years of age, multiple or recurrent pyogenic bacterial
infections (sepsis, pneumonia, meningitis, bone or joint infection, or abscess of
an internal organ or body cavity, but not otitis media or superficial skin or mucosal
abscesses) occurring two or more times in 2 years (for children age 13 and older,
see 114.00F4c); or
5. Multiple or recurrent bacterial infections, including pelvic inflammatory disease,
requiring hospitalization or intravenous antibiotic treatment three or more times
in a 12-month period.
OR
B. Fungal infections:
1. Aspergillosis; or
2. Candidiasis involving the esophagus, trachea, bronchi, or lungs, or at a site other
than the skin, urinary tract, intestinal tract, or oral or vulvovaginal mucous membranes;
or
3. Coccidioidomycosis, at a site other than the lungs or lymph nodes; or
4. Cryptococcosis, at a site other than the lungs (for example, cryptococcal meningitis);
or
5. Histoplasmosis, at a site other than the lungs or lymph nodes; or
6. Mucormycosis; or
7. Pneumocystis pneumonia or extrapulmonary Pneumocystis infection.
OR
C. Protozoan or helminthic infections:
1. Cryptosporidiosis, isosporiasis, or microsporidiosis, with diarrhea lasting for
1 month or longer; or
2. Strongyloidiasis, extra-intestinal; or
3. Toxoplasmosis of an organ other than the liver, spleen, or lymph nodes.
OR
D. Viral infections:
1. Cytomegalovirus disease (documented as described in 114.00F3b(ii)) at a site other than the liver,
spleen, or lymph nodes; or
2. Herpes simplex virus causing:
a. Mucocutaneous infection (for example, oral, genital, perianal) lasting for 1 month
or longer; or
b. Infection at a site other than the skin or mucous membranes (for example, bronchitis,
pneumonitis, esophagitis, or encephalitis); or
c. Disseminated infection; or
3. Herpes zoster:
a. Disseminated; or
b. With multidermatomal eruptions that are resistant to treatment; or
4. Progressive multifocal leukoencephalopathy.
OR
E. Malignant neoplasms:
1. Carcinoma of the cervix, invasive, FIGO stage II and beyond; or
2. Kaposi's sarcoma with:
a. Extensive oral lesions; or
b. Involvement of the gastrointestinal tract, lungs, or other visceral organs; or
3. Lymphoma (for example, primary lymphoma of the brain, Burkitt’s lymphoma, immunoblastic
sarcoma, other non-Hodgkin’s lymphoma, Hodgkin’s disease); or
4. Squamous cell carcinoma of the anal canal or anal margin.
OR
F. Conditions of the skin or mucous membranes (other than described in B2, D2, or D3,
above), with extensive fungating or ulcerating lesions not responding to treatment
(for example, dermatological conditions such as eczema or psoriasis, vulvovaginal
or other mucosal Candida, condyloma caused by human Papillomavirus, genital ulcerative disease).
OR
G. Neurological manifestations of HIV infection (for example, HIV encephalopathy, peripheral
neuropathy) resulting in one of the following:
1. Loss of previously acquired, or marked delay in achieving, developmental milestones
or intellectual ability (including the sudden onset of a new learning disability);
or
2. Impaired brain growth (acquired microcephaly or brain atrophy--see 114.00F4b);
or
3. Progressive motor dysfunction affecting gait and station or fine and gross motor
skills.
OR
H. Immune suppression and growth failure (see 114.00F4d) 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 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 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 weight-for-length table under
105.08B2.
I. Diarrhea, lasting for 1 month or longer, resistant to treatment and requiring intravenous
hydration, intravenous alimentation, or tube feeding.
OR
J. Lymphoid interstitial pneumonia/pulmonary lymphoid hyperplasia (LIP/PLH complex),
with respiratory symptoms that significantly interfere with age-appropriate activities,
and that cannot be controlled by prescribed treatment.
OR
K. One or more of the following infections (other than described in A-J, above). The
infection(s) must either be resistant to treatment or require hospitalization or intravenous
treatment three or more times in a 12-month period.
1. Sepsis; or
2. Meningitis; or
3. Pneumonia; or
4. Septic arthritis; or
5. Endocarditis; or
6. Sinusitis documented by appropriate medically acceptable imaging.
OR
L. Any other manifestation(s) of HIV infection, including those listed in 114.08A-K,
but without the requisite findings for those listings (for example, oral candidiasis
not meeting the criteria in 114.08F, diarrhea not meeting the criteria in 114.08I),
or other manifestation(s) (for example, oral hairy leukoplakia, hepatomegaly), resulting
in one of the following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age-group
criteria in paragraph B2 of 112.02.
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.
OR
D. Any other manifestation(s) of inflammatory arthritis resulting in one of the following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age-group
criteria in paragraph B2 of 112.02.
114.10 Sjögren’s syndrome. As described in 114.00D7. 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. Any other manifestation(s) of Sjögren’s syndrome resulting in one of the following:
1. For children from birth to attainment of age 1, at least one of the criteria in
paragraphs A-E of 112.12; or
2. For children age 1 to attainment of age 3, at least one of the appropriate age-group
criteria in paragraph B1 of 112.02; or
3. For children age 3 to attainment of age 18, at least two of the appropriate age.