Clinical and pathological studies on the autoimmune hepatitis and primary biliary cirrhosis overlap syndrome
- VernacularTitle:自身免疫性肝炎与原发性胆汁性肝硬化重叠综合征的临床与病理学研究
- Author:
Yanling SUN
;
Jingmin ZHAO
;
Xin MENG
- Publication Type:Journal Article
- Keywords:
autoimmune hepatitis and primary biliary cirrhosis overlap syndrome;
pathology, clinical
- From:
Medical Journal of Chinese People's Liberation Army
2001;0(07):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical and pathological features of the autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) overlap syndrome (AIH-PBC overlap syndrome). Methods The clinical data and liver biopsy specimens from 17 cases of the AIH-PBC overlap syndrome were analyzed and compared with 117 cases of AIH and 85 cases of PBC. The pathological features were as analyzed by histological observation and immunohistochemical staining for CK19, CD3, CD4, CD8, CD20, CD25, CD57 and CD68 in the biopsy liver tissues. Results Of the cases of autoimmune liver disease in this study, 8.4% were diagnosed as AIH-PBC overlap syndrome based on clinical, biochemical, serological and pathological examinations. Among the overlap syndrome cases, the females were in predominance (male∶female was 1∶8.5), and the median age of the patients presenting the clinical onset signs was 39.3 years, and the serological double-positive autoantibodies of ANA and AMA-M2 occupied 52.9%(9/17). Serum levels of ALT, ALP, ?-Glo, AST, TBIL, IgG and IgM were higher in patients with overlap syndrome than those in patients with AIH or PBC, respectively. The pathological findings in the biopsy liver tissues from the patients with overlap syndrome included moderate to severe interface hepatitis with plasma cell predominated mixed-inflammatory cells infiltration in hepatic and portal tracts, as well as the damage and obvious reactive proliferation of small bile ducts. Meanwhile, the amounts of CD3, CD4, CD8, CD20, CD25, CD57 and CD68 positive mononuclear cells increased in the overlap syndrome liver tissues compared with those in single PBC or AIH cases. Conclusion AIH-PBC overlap syndrome is not frequent (8.4%) in this study, but exist among autoimmune liver diseases in China. This overlap syndrome presented both clinical and pathological features of AIH and PBC, and cellular immune mediated injuries might be pivotal role in pathogenesis.
