Aleksandrova E A, Avdeev V G, Burnevich E Z, Arion E A
Ter Arkh. 2012;84(8):86-90.
Primary sclerosing cholangitis (PSC) is a chronic slowly progressive cholestatic liver disease characterized by non-purulent destructive inflammation and sclerosis of the extra- and intrahepatic bile ducts, leading to secondary biliary cirrhosis. The etiology of the disease is unknown; however, bacterial and genetic factors are presumed to be implicated. In 66-84% of cases, PSC is accompanied by inflammatory bowel diseases, such as ulcerative colitis and Crohn's disease. The diagnosis of PSC is based on the clinical presentation of the disease and on the data of magnetic resonance imaging or endoscopic retrograde cholangiography. The treatment of PSC has not been developed; there is evidence that ursodeoxycholic acid reduces cholestasis. Orthotopic liver transplantation is indicated at the stage of decompensated liver cirrhosis.
原发性硬化性胆管炎（PSC）是一种慢性、进展缓慢的胆汁淤积性肝病，其特征为肝内外胆管的非化脓性破坏性炎症和硬化，最终导致继发性胆汁性肝硬化。该病病因不明；然而，推测细菌和遗传因素与之有关。在66%-84%的病例中，PSC伴有炎症性肠病，如溃疡性结肠炎和克罗恩病。PSC的诊断基于该病的临床表现以及磁共振成像或内镜逆行胆管造影的数据。目前尚未开发出针对PSC的治疗方法；有证据表明熊去氧胆酸可减轻胆汁淤积。在失代偿期肝硬化阶段，建议进行原位肝移植。
