Predominant Features Associated with Systemic Sclerosis specific Autoantibodies

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Predominant Features Associated with Systemic Sclerosis specific Autoantibodies

From Steen, V. D. (2005). “Autoantibodies in systemic sclerosis.” Semin Arthritis Rheum 35(1): 35-42.

ACA aTh/ToU1-RNP (MCTD)PmSclU3-RNP (fibrillarin)Scl-70 (topoisomerase I)RNA Pol III b
SSc subset (% pts)Limited (50%–90%)Limited (4%–16%)Limited (100% MCTD)Limited (3%)Diffuse (7%)Diffuse (20%–30%)Diffuse (25%)
LungsPAHILD + PAHPAH
ILD
ILD
Myositis
ILD + PAHILD
KidneysSRCSRCSRC

ACA, anticentromere antibody; ILD, interstitial lung disease; MCTD, mixed connective tissue disease; PAH, pulmonary arterial hypertension; SRC, scleroderma renal crisis.

a ACA antibody positive patients should be screened for primary biliary cirrhosis with antimitochondrial antibody test and LFTs

b RNA polymerase III antibody positive patients are at higher risk for malignancy

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