Objective To further investigate the characteristic and the diagnostic value of the anti-nuclear antibody special fluorescence patterns in primary biliary cirrhosis. Methods The anti-nuclear antibody special fluorescence patterns in serum samples of 62 primary biliary cirrhosis ( PBC) and 515 control cases were tested by indirect immune fluorescence and the positive result were detected respectively by immune blotting assay. Results 49 of the 62 cases in primary biliary cirrhosis were positive for anti-nuclear antibody special fluorescence patterns, the sensibility is 79%,which was significantly higher than that in other control(P<0.05),and their specificity for PBC was 94.2%, moreover 83.7% showed high titer ( ≥1∶1000) .The target antigen in anti-nuclear antibody special mix fluorescence patterns was higher than that in special single fluorescence patterns,and the specificity in mix patterns was near to 100%. Conclusion The characteristic of the anti-nuclear antibody special fluorescence patterns has a close relation with primary biliiary cirrosis， especially the mix patterns of it has an important diagnostic value for PBC．