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).
(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
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,
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).
(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
(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
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).
(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.