原发性胆汁性胆管炎的诊疗进展

Advances in the diagnosis and management of primary biliary cholangitis

原发性胆汁性胆管炎(primary biliary cholangitis,PBC)是一种慢性自身免疫性肝内胆汁淤积性疾病,未经有效治疗可进展至肝硬化、肝衰竭,其诊断主要靠肝内胆汁淤积特征(碱性磷酸酶和谷氨酰转肽酶水平升高,影像学排除肝外胆系梗阻)、抗线粒体抗体阳性和/或以小叶间胆管非化脓性炎症为主的病理特征。新型特异性抗核抗体如抗-gp210和抗-sp100联合检测有助于诊断抗线粒体抗体阴性的PBC。熊去氧胆酸是治疗本病的首选药物,但30%~40%的患者生化应答欠佳,贝特类药物和奥贝胆酸可作为此类患者的二线治疗。

Primary biliary cholangitis(PBC)is an autoimmune liver disease characterized by chronic intrahepatic cholestasis.Without timely effective therapy,this disease may progress to cirrhosis and liver failure.The hallmark of PBC is cholestatic biochemistry and positive for anti-mitochondrial antibody(AMA)in the absence of extrahepatic obstruction.Specific antinuclear antibodies such as anti-gp210 and anti-sp100 can significantly improve the diagnosis of AMA-negative PBC.Ursodeoxycholic acid(UDCA)is the treatment of choice but 30%~40% of the patients show incomplete biochemical responses.The efficacy of the second-line therapy including fibrates and obeticholic acid can be used for patients who respond poorly to UDCA.