Objective To evaluate the clinical significance of antinuclear antibody (ANA) and ANA repertoire in the diagnosis of systemic sclerosis (SSc). Methods ANAs were tested in 64 pa tients with SSc and 30 healthy controls by applying indirect immunofluorescence (IIF) and immunoblotting (IB). Results IIF-ANA assay showed that the positive rate of ANAs was 98. 4% (63/64) in SSc, and ANA staining was mainly shown as homogeneous-nucleolar pattern; IB-ENA test indicated that the antinuclear antibodies were mainly anti-Scl-70, anti-SS-A and anti-nRNP in SSc, and the positive rate was respectively 65.1% (41/64), 29.7% (19/64) and 6.3% (4/64). Conclusion Existence of multiple antibodies in SSc patients may hint that the patients might have other autoimmune diseases or increased risk for other autoimmune diseases. Besides, IB assay significantly increases the sensitivity and specificity of ANAs measurement, and contributes to the diagnosis of SSc.