[Is autoimmune cholangitis-anti-mitochondrial antibody-negative primary biliary cholangitis still an independent disease?]

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.自身免疫性胆管炎（AIC）首次被报道于1987年，是一种慢性胆汁淤积性疾病，好发于中年女性，与原发性胆汁性胆管炎（PBC）有共同的临床表现、生物化学异常及病理学改变。但血清抗线粒体抗体（AMA）阴性，抗核抗体和/或平滑肌抗体阳性率较高。治疗上对熊去氧胆酸、类固醇的反应差，需加用免疫抑制剂治疗，且预后不佳。目前关于AIC确切病理机制尚不清楚，对其分类为新的一种自身免疫性肝病或AMA阴性PBC尚无统一论断。现通过检索归纳国内外有关AIC的文章进行分类总结，并与PBC进行对比。.