Stiehl A
Medizinische Universitätsklinik Heidelberg.
Internist (Berl). 2004 Jan;45(1):27-32. doi: 10.1007/s00108-003-1116-0.
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 belongs to the most common cholestatic diseases in adults and at present 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. Immunosuppressive treatment is little effective. Ursodeoxycholic acid (UDCA) has been shown to improve liver histology in PSC. 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 dilatation is highly effective. In patients with endstage 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，目前诊断频率不断增加。它常与溃疡性结肠炎相关。PSC患者胆管癌的发病率增加，溃疡性结肠炎患者结肠癌的发病率也增加。免疫抑制治疗效果不佳。熊去氧胆酸（UDCA）已被证明可改善PSC患者的肝脏组织学。目标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，主要胆管可能会出现狭窄，早期内镜扩张非常有效。对于终末期疾病患者，UDCA无效，需进行肝移植。
