Systemic Sclerosis (Scleroderma) 12 Antibodies Panel 2

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  Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, and cutaneous and visceral fibrosis. Some antibodies are considered highly specific for SSc,
including Scl-70 (anti-topoisomerase 1) antibody, anti-centromere (CENP) antibodies,
and anti-RNA polymerase III antibody and are classification criteria for disease
defined by the American College of Rheumatology. Centromere antibodies are
found in patients with a limited form of cutaneous systemic sclerosis, "CREST"
syndrome, a complex of subcutaneous calcinosis, Raynaud's phenomenon,
esophageal dysmotility, sclerodactyly, and telangiectasias. RNA polymerase III
antibodies target RNAP III epitopes 11 and 155.
Antibodies to three major components of U1-snRNP (snRNP RNP A,
U1-snRNP RNP C, U1-snRNP RNP-70kd): U1- are associated with scleroderma
and inflammatory myopathy overlap syndromes. Anti-fibrillarin (anti-U3RNP)
antibodies are associated with diffuse cutaneous SSc, frequent visceral involvement,
and especially renal and cardiac involvement. In African-American patients, anti-fibrillarin
antibodies are associated with severe pulmonary disease, pulmonary hypertension,
severe small bowel involvement, and a poor prognosis. Anti-Th/To antibodies primarily
bind to two proteins of the mitochondrial RNA processing (MRP) and the ribonuclease P complexes,
are present in 1-13% of SSc patients, and are rarely found in other diseases.
Anti-Th/To antibodies are primarily associated with localized cutaneous scleroderma,
and associated with pericarditis, interstitial lung disease and a high frequency
of “intrinsic pulmonary hypertension, and a poorer prognosis. Autoantibodies
to PM/Scl, the human exosome complex, are found in polymyositis/scleroderma
overlap syndromes. The majority of anti-PM/Scl reactivity is directed to one of
two proteins: PM/Scl100 and/or PM-Scl75.