Primary biliary cirrhosis (PBC) is, which a chronic, autoimmune liver disease. Some patients have antinuclear antibodies anti-Sp100, which are considered to be disease-specific. We compared an enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence (IIF) for detection of anti-Sp100. The sensitivity of anti-Sp100 determined by ELISA and IIF was 44% and 34%, respectively. Specificity was 99% for ELISA and 98% for IIF, respectively. The positive and negative predictive value (PPV, NPV) for anti-Sp100 determined by ELISA were 98%, 60% and 95%, 56% for IIF respectively. IIF required substantial experience in interpreting subjective patterns, whereas ELISA was more sensitive, cheaper, less time consuming, and produced clear-cut quantitative results.