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原发性硬化性胆管炎(PSC)是累及整个胆管系统,以闭塞性炎症与纤维化为特征的一种少见病。男性患者多,表现为进行性胆郁,肝内有较多铜的积聚,最终进展为肝硬化且早期出现肝功能衰竭。患者中54%～72%合并炎症性肠病,几平都是慢性溃疡性结肠炎(CUC)。但文献中同一家庭的成员发生PSC的报导仅有2个例子。在美国CUC的发病率为40～100/10万,14%患者合并肝胆系统疾病,但同一家庭的成员既有CUC又有PSC者尚未见报导。作者
Primary sclerosing cholangitis (PSC) is a rare disease characterized by occlusive inflammation and fibrosis involving the bile duct system. More male patients, manifested as progressive gallstones, the accumulation of more copper in the liver, the final progress of cirrhosis and early liver failure. 54% to 72% of patients with inflammatory bowel disease, a few are chronic ulcerative colitis (CUC). However, there are only two cases of PSC reported by members of the same family in the literature. The incidence of CUC in the United States is 40-100 / 100,000, 14% of patients with hepatobiliary diseases, but the same family members have both CUC and PSC have not been reported. Author