Mitchell S A, Chapman R W
Department of Gastroenterology, Oxford Radcliffe Hospital, UK.
Aliment Pharmacol Ther. 1997 Feb;11(1):33-43. doi: 10.1046/j.1365-2036.1997.91263000.x.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by a progressive obliterating fibrosis of the intrahepatic and extrahepatic bile ducts. The pathogenesis of PSC is poorly understood but it is thought to be an immune-mediated disease. The optimal therapy which successfully improves symptoms, delays progression towards liver failure and transplantation and prevents the onset of cholangiocarcinoma remains elusive. Although current treatments are used to manage cholestasis and its consequences and some of the more general complications of the disease, none of the current therapeutic agents have been shown to retard and reverse the rate of disease progression. The role of cupruretics, corticosteroids, methotrexate, antifibrogenic agents and ursodeoxycholic acid in the treatment of PSC is reviewed. Orthotopic liver transplantation remains the only therapeutic option for advanced PSC but the timing of transplantation remains controversial and the possibility of recurrence of the disease in the graft is increasingly recognised. It is likely that greater insight into the pathogenetic mechanisms involved in PSC will allow therapy to be targetted more specifically at the biliary epithelium.
原发性硬化性胆管炎（PSC）是一种慢性胆汁淤积性肝病，其特征为肝内和肝外胆管进行性闭塞性纤维化。PSC的发病机制尚不清楚，但被认为是一种免疫介导的疾病。能成功改善症状、延缓向肝衰竭和肝移植发展以及预防胆管癌发生的最佳治疗方法仍未找到。尽管目前的治疗方法用于处理胆汁淤积及其后果以及该疾病的一些更常见并发症，但目前尚无治疗药物被证明能延缓和逆转疾病进展速度。本文综述了铜螯合剂、皮质类固醇、甲氨蝶呤、抗纤维化药物和熊去氧胆酸在PSC治疗中的作用。原位肝移植仍然是晚期PSC的唯一治疗选择，但移植时机仍存在争议，并且越来越认识到疾病在移植物中复发的可能性。对PSC发病机制的更深入了解可能会使治疗更有针对性地作用于胆管上皮。
