Abstract: Objective The aim of this study was to investigate diagnostic performance of Paris criteria in Chinese patients with autoimmune hepatitis/primary biliary cholangitis overlap syndrome（AIH/PBC OS）.Methods We retrospectively analyzed the clinical data of 24 patients with PBC and 16 with AIH/PBC OS diagnosed based on Paris' criteria,and 14 patients with PBC underwent liver biopsies.Results Serum bilirubin,ALT,GGT and IgG levels in 16 patients with AIH/PBC OS were（45.3±48.3） μmol/L,（236.8±71.8） U/L,（305.3±258.5） U/L and （25.3±9.9） g/l,significantly different as compared to【（53.4±57.8） μmol/L,（77.5±71.7） U/L,（389.2±324.3） U/L and （13.9±6.0） g/l,respectively,P<0.05】 in 24 patients with PBC; the prevalence of serum ANA,AMA and AMA-M2,but SMA（43.8% in patients with AIH/PBC OS vs. 4.2% in patient with PBC） between the two groups were not significantly different（P>0.05）;we modified five patients' diagnosis from PBC to AIH/PBC OS as the liver histopathological evidence proved,while serum ALT levels in them were 40.0U/L,82.8U/L,94.1 U/L,162.1 U/L and 117.2 U/L,much lower than 5×ULN. Conclusion For the diagnosis of Chinese patients with AIH/PBC OS,we strongly recommend liver biopsy in order not to miss diagnosis.

Key words: Autoimmune hepatitis/Primary biliary cholangitis overlap syndrome, Primary biliary cholangitis, Paris' criteria, Diagnosis