Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are rare, chronic cholestatic diseases that can progress to liver failure. The goals of treatment are to halt the progression of liver disease to cirrhosis and/or liver failure, and alleviate symptoms associated with these diseases. Ursodeoxycholic acid (UDCA) has historically been the first-line treatment for PBC, with obeticholic acid (OCA) and fibrates used as second-line or adjunctive therapies. However, the treatment landscape is rapidly expanding. Recently, two new second line agents gained FDA approval for the treatment of PBC, and several other therapies remain under investigation with promising results. While significant progress has been made in development of therapies for PBC, there are no current approved treatments for PSC other than liver transplantation although several emerging therapies have shown encouraging results. This review outlines the current and upcoming treatments for PBC and PSC.

中文翻译：

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原发性胆汁性胆管炎和原发性硬化性胆管炎治疗景观。


原发性胆汁性胆管炎 （PBC） 和原发性硬化性胆管炎 （PSC） 是罕见的慢性胆汁淤积疾病，可进展为肝功能衰竭。治疗目标是阻止肝病进展为肝硬化和/或肝衰竭，并缓解与这些疾病相关的症状。熊去氧胆酸 （UDCA） 历来是 PBC 的一线治疗，奥贝胆酸 （OCA） 和贝特类药物用作二线或辅助治疗。然而，治疗前景正在迅速扩大。最近，两种新的二线药物获得了 FDA 的批准，用于治疗 PBC，其他几种疗法仍在研究中，结果令人鼓舞。虽然 PBC 疗法的开发取得了重大进展，但目前没有批准的 PSC 治疗方法，除了肝移植，尽管几种新兴疗法已显示出令人鼓舞的结果。本综述概述了 PBC 和 PSC 当前和即将进行的治疗方法。