Abstract: Objective The aim of this study was to investigate clinical feature, serum autoantibody profiles and prognosis of patients with primary biliary cholangitis (PBC). Methods 60 patients with PBC were encountered in our hospital between March 2020 and December 2024, and all received long-term oral ursodeoxycholic acid (UDCA) treatment. Serum IgM, autoantibody profiles were detected routinely, and liver stiffness measurement (LSM) was measured by Fibroscan. Results As for initial symptoms in our 60 patients with PBC, fatigue accounted for 46.7%, itching for 31.7%, jaundice for 21.7%, right upper quadrant discomfort for 11.7%, ascites for 5.0%, splenomegaly for 3.3% and gastrointestinal symptoms for 5.0%, and concomitant with SjÏgren's syndrome for 23.3%, systemic lupus erythematosus for 6.7% and rheumatoid arthritis for 5.0%; serum AMA-M2 positive rate accounted for 91.7%, AMA for 81.7%, anti-SLA/LP for 3.3%, anti-LKM-1 for 1.7%, anti-gp210 for 26.7%, anti-Sp100 for 15.0% and anti-LC-1 for 3.3%; serum ALP, total serum bilibubin, IgM, LSM and Mayo’s score in 16 patients with serum anti-gp210 positive were (295.0±82.5)U/L, (24.5±10.3)μmol/L, (4.2±1.1)g/L, (14.1±3.8)kPa and (5.9±1.2), all significantly higher than [(210.5±65.8)U/L, (15.7±7.6)μmol/L, (3.7±1.0)g/L, (10.8±3.0)kPa and (4.7±1.0), respectively, P<0.05], while complete response rate to UDCA therapy was 43.7%, significantly lower than 77.3%(P<0.05) in 44 patients with serum anti-gp210 negative; by end of medium 26 month of follow-up, the incidences of liver cirrhosis, decompensated liver functions and liver transplantation/death in patients with serum anti-gp210 positive were 43.7%, 37.5% and 18.7%, all much higher than 20.4%, 11.4% and 4.5%(P<0.05) in those with serum anti-gp210 negative. Conclusion Serum anti-gp210 antibody positive might hints even more clinical severity of disease, and clinicians should take more attention to them.

Key words: Primary biliary cholangitis, Anti-gp210 antibody, Anti-mitochondrial antibody, Ursodeoxycholic acid, Therapy