原发性硬化性胆管炎(PSC)是一种原因不明的慢性胆汁淤积性肝脏疾病,特点是肝内、外胆道系统的炎症、纤维化和破坏,最后可以导致胆汁性肝硬化。组织特点是胆管节段性扩张和狭窄。大多数情况下病程进展缓慢,有时也非常快,最终发展为胆汁性肝硬化。除了自身免疫性肝炎和原发性胆汁性硬化外,PSC也算一种肝自身免疫性疾病。 每100000人中约有1～8人发生PSC。其中70％为男性。平均诊断年龄为39岁。约75％的病人有慢性肠炎,其中87％为溃疡性结肠炎,13％为 Primary sclerosing cholangitis (PSC) is an unidentified chronic cholestatic liver disease characterized by inflammation, fibrosis and destruction of the intrahepatic and extrabiliary biliary systems that can eventually lead to biliary cirrhosis. Tissue is characterized by segmental dilatation and stenosis. In most cases, the course of disease progresses slowly, sometimes very fast, and eventually develops into biliary cirrhosis. In addition to autoimmune hepatitis and primary biliary cirrhosis, PSC is also considered a liver autoimmune disease. Approximately 1 to 8 people develop PSC per 100,000 people. 70% of them are men. The average diagnosis is 39 years old. About 75% of patients have chronic enteritis, of which 87% are ulcerative colitis and 13% are