原发性胆汁性肝硬化 (ＰＢＣ)是一种原因不明的自身免疫性疾病 ,主要表现为小叶内和间隔胆管的进行性炎症性破坏 ,伴门静脉周围炎症 ,继而纤维化 ,最后演变为肝硬化 ,最终引起门静脉高压及终末期肝病的其它并发症而导致死亡。本病呈世界性分布 ,发病率约为每百万人口中 40～15 Primary biliary cirrhosis (PBC) is an unidentified autoimmune disease characterized mainly by progressive inflammatory destruction of the interlobular and septal bile ducts accompanied by inflammation of the periphery of the portal vein, followed by fibrosis, which eventually leads to cirrhosis Eventually leading to other complications of portal hypertension and end-stage liver disease leading to death. The disease is worldwide distribution, the incidence of about 40 to 15 per million population