Abstract: Objective To explore the biochemical-immune and pathological characteristics of autoimmune hepatitis（AIH） with Sj?gren’s syndrome （SS）. Methods A total of 76 cases of AIH patients were included from January 2009 to April 2017. Among them, there were 40 cases of AIH with SS and 36 cases without SS. The liver function, immunological index, histological features, length of first diagnosis and treatment costs were compared between the two groups. ResultsFor AIH+SS group and AIH group, the proportion of women were 97.5% and 77.8%, the proportion of the first diagnosis age less than 60 years were 70% and 47.2%, the median course of disease were 30 months and 9 months, all the difference were statistically significant (P<0.05). The chief complaints in AIH+SS group and AIH group were as follows: cutaneous or scleracterus (52.5% vs. 38.9%), abnormal transaminase (17.5% vs. 44.4%), dryness of mouth and eye (15.0% vs. 2.8%), all the difference were statistically significant (P<0.05). There were no statistically significant difference in hospitalization expenses, and length of stay between the two groups (P>0.05). The median level of total bilirubin (TBIL), direct bilirubin (DBIL) and immunoglobulin (Ig) M of AIH +SS group were higher than those of AIH group, the mean level of albumin (ALB) and complement 3 （C3） of AIH +SS group were lower than those of AIH group, and the positive rate of anti-mitochondrial antibody-M2 （AMA-M2）, anti-Ro antibody A (SSA), anti-La antibody (SSB) and anti-soluble liver antigen antibody (SLA) of AIH+SS group were higher than those of AIH group (P<0.05). There were no statistically significant difference in histological changes of hepatocytes and bile duct injury rate (P>0.05). Conclusion AIH patients in young and middle-aged women need to be vigilant with SS with main manifestation of skin sclera and high specific autoantibodies positive.