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自身免疫性肝炎的重叠综合征:一个悬而未决的问题。

Overlap syndromes of autoimmune hepatitis: an open question.

机构信息

Department of Internal Medicine, University of Turin, C.so A.M. Dogliotti 14, 10126, Turin, Italy.

出版信息

Dig Dis Sci. 2013 Feb;58(2):344-8. doi: 10.1007/s10620-012-2378-y. Epub 2012 Oct 21.

Abstract

The headword "overlap syndromes" of liver diseases includes the coexistence of autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. These syndromes often represent a diagnostic and therapeutic challenge for hepatologists; it remains unclear whether these overlap syndromes form distinct entities or they are only variants of the major autoimmune liver diseases. The most frequent reported association occurs between autoimmune hepatitis and primary biliary cirrhosis, whereas the overlap between autoimmune hepatitis and primary sclerosing cholangitis is less frequent, typically at young age and often attendant with an inflammatory bowel disease. The choice therapy is based on ursodeoxycholic acid and immunosuppressive drugs, used at the same time or consecutively, according to the course of disease. The diagnostic scores for autoimmune hepatitis can help for diagnosis, even though their definitive soundness is lacking.

摘要

肝脏疾病的“重叠综合征”包括自身免疫性肝炎、原发性胆汁性肝硬化和原发性硬化性胆管炎的共存。这些综合征常常给肝病学家带来诊断和治疗方面的挑战;目前尚不清楚这些重叠综合征是否构成不同的实体,或者它们是否只是主要自身免疫性肝病的变异。最常报道的关联发生在自身免疫性肝炎和原发性胆汁性肝硬化之间,而自身免疫性肝炎和原发性硬化性胆管炎之间的重叠则较为少见,通常发生在年轻患者中,常伴有炎症性肠病。根据疾病的进程,选择熊去氧胆酸和免疫抑制剂联合或序贯治疗。自身免疫性肝炎的诊断评分有助于诊断,尽管其准确性尚缺乏。

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