Department of Gastroenterology and Hepatology, Tytgat Institute for Liver and Intestinal Research, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
Liver Int. 2017 Jan;37 Suppl 1:123-129. doi: 10.1111/liv.13306.
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic (PBC) or larger intra- and/or extrahepatic (PSC) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified. Ursodeoxycholic acid (UDCA) is the standard treatment of PBC and is used also for other cholestatic conditions including PSC, and it exerts anticholestatic effects at adequate doses. Novel anticholestatic therapeutic options for patients not adequately responding to UDCA are under development or have, like obeticholic acid, already been proven to have efficacy when combined with UDCA in the treatment of PBC. The future role of immunomodulating/immunosuppressive drug regimens must be critically reviewed.
原发性胆汁性胆管炎 (PBC) 和原发性硬化性胆管炎 (PSC) 是最常见的慢性胆汁淤积性肝病，作为 2017 年讨论胆汁淤积症管理的模型疾病在本报告中进行了总结。PBC 和 PSC 的特征是肝内小胆管（PBC）或较大的肝内和/或肝外胆管（PSC）的炎症和纤维化。胆管损伤导致胆汁淤积，并可进展为肝纤维化甚至肝硬化。各种遗传、环境和内源性因素可能导致慢性胆汁淤积性肝病的发生，但 PBC 和 PSC 的确切发病机制尚未阐明。熊去氧胆酸 (UDCA) 是 PBC 的标准治疗药物，也用于包括 PSC 在内的其他胆汁淤积症，在足够剂量下发挥抗胆汁淤积作用。对于对 UDCA 反应不足的患者，正在开发新的抗胆汁淤积治疗选择，或者像奥贝胆酸一样，已经被证明在联合 UDCA 治疗 PBC 时具有疗效。免疫调节/免疫抑制药物方案的未来作用必须进行严格审查。
