Serum autoantibodies in scleroderma

Antigen	ANA staining pattern	Approximate frequency in all patients (%)	Clinical associations	Organ involvement
Scl-70 (topoisomerase-1)	Speckled	10 to 40	dcSSc	Lung fibrosis, isolated pulmonary hypertension less likely
Centromere	Centromere (kinetochore)	15 to 40	lcSSc	Pulmonary hypertension, esophageal disease, "protection" from lung fibrosis and kidney disease
RNA polymerase III	Fine speckled nucleolar	4 to 25	dcSSc	Kidney, skin, malignancy
U3 RNP (fibrillarin)	Nucleolar	1 to 5	dcSSc, poor outcome, Black males	Pulmonary hypertension, muscle
PM-Scl	Nucleolar	3 to 6	Overlap, mixed	Muscle
U1 RNP	Speckled	5 to 35	lcSSc, Black patients, polymyositis overlap	Muscle
Th/To	Nucleolar	1 to 7	lcSSc	Pulmonary hypertension, lung fibrosis, small bowel
Anti-U11/U12	Nucleolar	1 to 5	lcSSc and dcSSc	Lung fibrosis
Anti-Ku		1 to 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.

ANA: antinuclear antibody; dcSSc: diffuse cutaneous systemic sclerosis; lcSSc: limited cutaneous systemic sclerosis; SLE: systemic lupus erythematosus.

Adapted from: Nihtyanova SI, Denton CP. Autoantibodies as predictive tools in systemic sclerosis. Nat Rev Rheumatol 2010; 6:112.

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Serum autoantibodies in scleroderma