Is autoimmune cholangitis-anti-mitochondrial antibody-negative primary biliary cholangitis still an independent disease?
10.3760/cma.j.issn.1007-3418.2019.05.015
- VernacularTitle: 自身免疫性胆管炎—抗线粒体抗体阴性原发性胆汁性胆管炎还是独立的疾病？
- Author:
Jiandan QIAN
1
;
Guiqiang WANG
Author Information
1. Department of Infectious Disease, Peking University First Hospital, Beijing 100034, China
- Publication Type:Review
- Keywords:
Autoimmune cholangitis;
Primary biliary cholangitis;
Anti-mitochondrial antibodies;
Anti-gp210 antibody;
Anti-sp100 antibody
- From:
Chinese Journal of Hepatology
2019;27(5):393-396
- CountryChina
- Language:Chinese
-
Abstract:
Autoimmune cholangitis (AIC) was first reported in 1987 as a chronic cholestatic disease that occurs predominantly in middle-aged women and has a common clinical manifestations, biochemical abnormalities and pathological changes with primary biliary cholangitis (PBC). However, serum anti-mitochondrial antibodies (AMA) are negative, and ANA and/or smooth muscle antibody positive rates are higher. The treatment response and prognosis with ursodeoxycholic acid and steroids is poor, thus it needs to be treated with immunosuppressive agents. Presently, the exact pathological mechanism of AIC is still unclear, and there is no unified assertion that classifies it as a new autoimmune liver disease or AMA-negative PBC. This article reviews the worldwide published work on AIC and compares them with PBC.
