Analysis of Clinical Characteristics of Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlap Syndrome / 胃肠病学

ABSTRACT

Background:The overlap of autoimmune liver disease with extrahepatic autoimmune disease adds the difficulty of diagnosis. It is essential to understand their clinical features. Aims:To analyze the clinical characteristics of primary biliary cirrhosis-autoimmune hepatitis( PBC-AIH ) overlap syndrome, and to investigate the effect of extrahepatic autoimmune disease on PBC-AIH. Methods:The clinical data including general information,clinical manifestations, biochemical parameters,immunological parameters,liver histopathological features,imaging findings and incidence of extrahepatic autoimmune disease of 81 patients with PBC-AIH were retrospectively analyzed. According to whether overlapping with extrahepatic autoimmune disease,PBC-AIH patients were divided into combination group and non-combination group. The clinical characteristics of the two groups were analyzed. Results:The ratio of male to female of 81 patients with PBC-AIH was 1 ∶ 9. 1,and the average age was(58. 1 ± 9. 9)years old. Of the patients 53. 1% had symptoms,the percentage of jaundice and pruritus was 18. 5% . Serum levels of ALT,AST,ALP,GGT,TBil and Glo were increased in all patients and IgG and IgM levels were increased in most of the patients. The positive rates of ANA, AMA and/ or AMA-M2,SMA and gp210 and/ or sp100 were 100% ,90. 1% ,1. 2% and 11. 1% ,respectively. Portal lymphoplasmacyte infiltration and interface hepatitis were the major liver histopathological features;50. 6% of the patients had both histological features of PBC and AIH. Cirrhosis was diagnosed in 28 patients(34. 6% )by imaging,and abdominal lymph nodes enlargement was found in 76 patients(93. 8% ). Twenty-three patients(28. 4% )overlapped with extrahepatic autoimmune disease;Sjogren’s syndrome and autoimmune thyroid disease were the most frequently overlapped extrahepatic autoimmune diseases. The level of GGT was significantly lower in combination group than in non-combination group(P = 0. 001). No statistically significant differences in other clinical characteristics were found between the two groups. Conclusions:It is crucial to understand the clinical characteristics of PBC-AIH and to screen the overlapped extrahepatic autoimmune disease,especially Sjogren’s syndrome and autoimmune thyroid disease.