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©2013 UpToDate ® Print Email Serum autoantibodies in scleroderma Antigen ANA staining pattern Approximate frequency in all patients (percent) Clinical associations Organ involvement Scl-70 (topoisomerase-1) Speckled 10-40 dcSSc Lung fibrosis, isolated pulmonary hypertension less likely RNA polymerase III Fine speckled nucleolar 4-25 dcSSc Renal, skin, pulmonary hypertension U3 RNP (fibrillarin) Nucleolar 1-5 dcSSc, poor outcome black men Pulmonary hypertension, muscle PM-Scl Nucleolar 3-6 Overlap, mixed Muscle U1 RNP Speckled 5-35 lcSSc, blacks, polymyositis overlap Muscle Centromere Centromere (kinotochore) 15-40 lcSSc Pulmonary hypertension, esophageal disease, "protection" from lung fibrosis and renal disease Th/To Nucleolar 1-7 lcSsc Pulmonary hypertension, lung fibrosis, small bowel Anti U11/U12 Nucleolar 1-5 lcSSc and dcSSc Lung fibrosis Anti-Ku 1-3 Overlap SSc Muscle and joint involvement, SLE overlap Characteristics and clinical associations of the different autoantibodies that may be seen in scleroderma. dcSSc and lcSSc refer to diffuse and limited cutaneous systemic sclerosis, respectively. Adapted from: Nihtyanova SI, Denton CP. Autoantibodies as predictive tools in systemic sclerosis. Nat Rev Rheumatol 2010; 6:112.