Objective To explore the prevalence of autoimmune liver disease-related antibodies in patients with PBC, and study the clinic significance of autoimmune liver disease related antibody profiles in patients with PBC. Methods The anti-AMA in 247 specimens from patients with liver disease, including 173 PBC, 37 AIH and 37 LDC were detected by IIF. Anti-AMA-M2, anti-GP210, anti-SP100, anti-SLA, anti-LCI and anti-LKM-1 antibodies were measured by ELISA. Results The positive rates of anti-AMA, anti-AMA-M2, anti-GP210, anti-SPl00, anti-LC1, anti-SLA and anti-LKM-1 antibodies were92. 5% (160/ 173), 86.7% (150/173), 35. 8% (62/173), 24. 3% (42/173), 0.6% (1/173), 0% (0/173) and 0.6%(l/173) in PBC group, 18.9% (7/37), 5.4% (2/37),8.1% (3/37),13. 5% (5/37),0% (0/ 37 ) ,5.4% (2/37 ) and 2. 7% (1/37 ) in AIH group and 5.4% (2/37), 2.7% (1/37 ) , 5.4% (2/37 ) , 10. 8% (4/37) , 0% (0/37) , 0% (0/37) and 0% (0/37) respectively in LDC group. Anti-AMA, anti-AMA-M2 and anti-GP210 was detected more frequently in patients with PBC group than AIH group (x~2 =101.3,100.8 and 11.0,P<0.01) while anti-SLA was detected more frequently in patients with AIH group than PBC group (x~2 = 9. 4, P < 0.01). The levels of ALT, TBIL, DBIL, GGT and ALP were higher in patients known to have positive anti-GP210 ( U = 1212.0,1199.0,1218.0,1074.0,1030. 0,P < 0. 01) and the levels of IgM were higher in patients known to have positive AMA ( U = 94.0, P <0.05). Conclusions Anti-LCI, anti-SLA and anti-LKM-1 antibodies in PBC and AIH are detected at a very low frequency in the corhort. Anti-GP210 antibody is found to be associated with the severity of liver damage while AMA is found to be associated with immunologic function in patients with PBC. There is little significance for screening anti-LCI, anti-SLA, anti-LKM-1 antibodies in patients with autoimmune liver diseases. It is of importance to detect anti-AMA and anti-GP210 antibodies for diagnosis of PBC.