Stiehl A, Benz C, Sauer P
Medizinische Universitätsklinik, Heidelberg, Germany.
Can J Gastroenterol. 2000 Apr;14(4):311-5. doi: 10.1155/2000/983681.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by fibrosing inflammation and obliteration of intra- and/or extrahepatic bile ducts. The disease is one of the most common cholestatic diseases in adults and is diagnosed with increasing frequency. It is very often associated with ulcerative colitis. Patients with PSC have an increased incidence of bile duct carcinomas, and those with ulcerative colitis also have an increased incidence of colonic carcinomas. In end-stage disease, liver transplantation is the treatment of choice. Immunosuppressive treatment has little effect. Ursodeoxycholic acid (UDCA), which has been shown to improve liver histology and survival in patients with primary biliary cirrhosis, has a beneficial effect in PSC, provided that patients who develop major duct stenoses are treated endoscopically. The aim is to treat patients as early as possible to prevent progression to the advanced stages of the disease. During treatment with UDCA, stenoses of major ducts may develop, and early endoscopic dilation is highly effective. Because UDCA treatment improves but does not cure cholestatic liver diseases, permanent treatment seems to be necessary. Such prolonged treatment with UDCA may be recommended because, until now, no side effects have been reported. In patients with end-stage disease, UDCA is not effective and liver transplantation is indicated.
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/或肝外胆管的纤维化炎症和闭塞。该疾病是成人中最常见的胆汁淤积性疾病之一，诊断频率日益增加。它常与溃疡性结肠炎相关。PSC患者胆管癌的发病率增加，而溃疡性结肠炎患者结肠癌的发病率也增加。在终末期疾病中，肝移植是首选治疗方法。免疫抑制治疗效果甚微。熊去氧胆酸（UDCA）已被证明可改善原发性胆汁性肝硬化患者的肝脏组织学并提高生存率，在PSC中也有有益作用，但前提是发生主要胆管狭窄的患者要接受内镜治疗。目的是尽早治疗患者以防止疾病进展至晚期。在使用UDCA治疗期间，可能会出现主要胆管狭窄，早期内镜扩张非常有效。由于UDCA治疗可改善但不能治愈胆汁淤积性肝病，似乎需要长期治疗。可能推荐使用UDCA进行这种长期治疗，因为到目前为止尚未报告有副作用。对于终末期疾病患者，UDCA无效，应进行肝移植。
