Worthington Joy, Chapman Roger
Department of Gastroenterology, John Radcliffe Hospital, Headington, Oxford, OX3 9DU, UK.
Orphanet J Rare Dis. 2006 Oct 24;1:41. doi: 10.1186/1750-1172-1-41.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9-1.31/100,000 and point prevalence of 8.5-13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8-30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.
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，其特征为胆管树的炎症和纤维化。诊断时的平均年龄为40岁，男性受影响的频率是女性的两倍。据报道，PSC的年发病率为0.9 - 1.31/100,000，点患病率为8.5 - 13.6/100,000。PSC的发病通常隐匿，许多患者在诊断时无症状或仅有轻微症状，如疲劳、腹部不适和瘙痒。在疾病后期，脾肿大和黄疸可能是其特征。在大多数情况下，疾病会进展为肝硬化和肝衰竭。8% - 30%的患者会发生胆管癌。PSC被认为是免疫介导的，常与炎症性肠病，尤其是溃疡性结肠炎相关。该疾病根据典型的胆管造影和组织学表现，并在排除继发性硬化性胆管炎后进行诊断。有症状患者自诊断起的中位生存期估计为12年。诊断时无症状的患者，其中大多数会发展为进行性疾病，诊断后16年的生存率大于70%。肝移植仍然是PSC终末期肝病患者唯一有效的治疗选择，尽管高剂量熊去氧胆酸可能有有益作用。
