Virović Lucija, Supanc Vladimir, Duvnjak Marko
Zavod za gastroenterologiju i hepatologiju Klinika za unutarnje bolesti Klinicka bolnica Sestre milosrdnice Vinogradska 29, 10000 Zagreb, Hrvatska.
Acta Med Croatica. 2003;57(3):207-19.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin, characterized by inflammation, fibrosis, and obliteration of bile ducts, which ultimately results in biliary cirrhosis. The condition most commonly affects intrahepatic and extrahepatic bile ducts together, but sometimes only intrahepatic or extrahepatic ducts are involved. PSC is often associated with inflammatory bowel disease, especially ulcerative colitis. The majority of patients are initially asymptomatic, and identified on the basis of elevated serum levels of alkaline phosphatase or gamma-glutamyl transpeptidase, especially while screening patients with ulcerative colitis. Diagnosis is based on characteristic cholangiographic appearance with focal bile duct dilatations proximal to areas of stricturing that produce a beaded appearance. Ursodeoxycholic acid is most effective medical therapy, with other symptomatic measures, while liver transplantation is the treatment of choice for patients with advanced liver disease.
原发性硬化性胆管炎（PSC）是一种病因不明的慢性胆汁淤积性肝病，其特征为胆管炎症、纤维化和闭塞，最终导致胆汁性肝硬化。该病最常同时累及肝内和肝外胆管，但有时仅累及肝内或肝外胆管。PSC常与炎症性肠病相关，尤其是溃疡性结肠炎。大多数患者最初无症状，是在血清碱性磷酸酶或γ-谷氨酰转肽酶水平升高时被发现的，特别是在筛查溃疡性结肠炎患者时。诊断基于特征性胆管造影表现，即狭窄部位近端的局灶性胆管扩张，呈现串珠样外观。熊去氧胆酸是最有效的药物治疗方法，同时配合其他对症措施，而肝移植是晚期肝病患者的首选治疗方法。
