Michaels Anthony, Levy Cynthia
Division of Gastroenterology, Hepatology, and Nutrition, University of Florida-Gainesville, USA.
Medscape J Med. 2008 Mar 12;10(3):61.
Primary sclerosing cholangitis (PSC) is a rare, chronic cholestatic liver disease of uncertain etiology characterized by the destruction of the intrahepatic and/or extrahepatic ducts through inflammation and fibrosis. This ultimately leads to biliary complications including cirrhosis, cholangiocarcinoma, and eventually death. Given the uncertainty surrounding the pathogenesis of the disease, a number of different medical therapies have been studied in the treatment of PSC. However, there currently are no effective medical therapies known to halt the progression of disease. The only definitive therapy for PSC is liver transplantation. This review will primarily focus on the medical approaches that have been studied for the treatment of PSC as well as on the management of symptoms commonly associated with the disease.
原发性硬化性胆管炎(PSC)是一种病因不明的罕见慢性胆汁淤积性肝病,其特征是肝内和/或肝外胆管通过炎症和纤维化被破坏。这最终会导致包括肝硬化、胆管癌等在内的胆道并发症,最终导致死亡。鉴于该疾病发病机制尚不确定,人们对多种不同的药物治疗方法进行了研究以治疗PSC。然而,目前尚无已知的有效药物治疗方法能够阻止疾病进展。PSC唯一的确定性治疗方法是肝移植。本综述将主要关注已研究的治疗PSC的医学方法以及该疾病常见症状的管理。