Abstract: Objection  To summary and analyze the clinic characteristics features and associated extrahepatic autoimmune diseases of patients with primary biliary cirrhosis -autoimmune hepatitis overlap syndrome(AIH-PBC OS). Methods   To analyze retrospectively the clinic characteristics and pathologic features of 49 patients that were diagnosed as PBC-AIH OS and been followed-up when been treated in digestive department of general hospital during Jan.2000 to Jan.2012. Results   These patients were most female with age of 51-60 years old, and the mean age of onset was 57.2±8.9 years old. The levels of serum ALT, AST, GGT and TBIL were increased in various degree in all the patients, while serum GLO/IgG was inceased in 79.6% patients. Positive rate of ANA,AMA and SMA was 98.0%,89.8% and 6.1% respectively. When been diagnosed, 38.8% of the patients had been developed to hepatic cirrhosis, 57.9%(11/19)of them at the stage of decompensation . Besides, pathologic stage was 3,4 in 16 (66.7%)patients with PBC and 1,2 stage in 8(33.3%)ones. Among them, there were 20 (40.8%)patients had extrahepatic autoimmune diseases at the same time, 16 cases (32.7%) had two kinds of autoimmune disease, 4 cases (8.1%) with three kinds of autoimmune diseases. Abdominal ultrasonic wave showed abnormality in 93.9% of patients,the abdominal lymph nodes were enlarged in 91.8% of the patients. Conclusion  Most the patients with PBC-AIH OS had been diagnosed as hepatic cirrhosis at the first time, so as to make early diagnosis and treatment. The symptoms were various and complicated so that it’s significant to expand the screening of incorporated autoimmune diseases. Abdominal lymph nodes enlargerment found by imaging examination may had guiding sense to diagnosis.

Key words: hepatitis, autoimmune diseases, hepatic cirrhosis, cholestasis, Overlap Syndrome, 病理学