原发性硬化性胆管炎的诊治进展

An Update on Diagnosis and Management of Primary Sclerosing Cholangitis

原发性硬化性胆管炎（PSC）是一种病因不明的自身免疫性疾病，诊断多依赖于影像学的特征性改变，如胆管呈串珠样改变。PSC的治疗效果欠佳，多推荐高剂量熊去氧胆酸，然而尚缺乏证据显示其可逆转病程转归，目前已知惟一有效的治疗方法为肝移植。PSC常因发生肝硬化和胆管癌而预后欠佳。本文对PSC的诊治进展作一概述。

Primary sclerosing cholangitis （PSC） is an autoimmune disease with unknown etiology, its diagnosis usually depends on characteristic imaging findings, including segmental fibrosis of bile ducts with saccular dilatations between strictures. Current medical therapies are unsatisfactory. High-dose ursodeoxycholic acid has been recommended, however, evidence showing delay or reversal of the natural history of disease is still lacking. Liver transplantation continues to be the only option with known therapeutic benefit. Patients with PSC suffer poor prognosis due to the occurrence of cirrhosis and cholangiocarcinoma. The present review addressed the recent achievements on the diagnosis and management of PSC.