Norwegian PSC Research Center, Clinic for Specialized Medicine and Surgery, Oslo University Hospital, Rikshospitalet, 0027 Oslo, Norway.
Best Pract Res Clin Gastroenterol. 2010 Oct;24(5):655-66. doi: 10.1016/j.bpg.2010.07.005.
Primary sclerosing cholangitis (PSC) is a chronic bile duct disease leading to fibrotic biliary strictures and liver cirrhosis. The patient population is heterogeneous with regard to disease progression and the presence of co-morbidities, complicating the practical handling of patients as well as studies of pathogenetic mechanisms. The aetiology of PSC is unknown, but the recent findings of several robust susceptibility genes emphasise the importance of genetic risk factors. There is no effective medical treatment available to delay the disease progression, but endoscopic therapy of biliary stenoses may be indicated. Follow-up of patients includes management of the inflammatory bowel disease that is found in the majority of cases along with investigations aimed at the early detection of cholangiocarcinoma and colorectal cancer, which also occur at increased frequencies. In the present review, we aim to summarise the present knowledge of PSC with a particular emphasis on the possible basis of disease variability.
原发性硬化性胆管炎（PSC）是一种慢性胆管疾病，可导致纤维性胆管狭窄和肝硬化。由于疾病进展和合并症的存在，患者群体存在异质性，这增加了患者实际处理和发病机制研究的复杂性。PSC 的病因尚不清楚，但最近发现的几个强有力的易感基因强调了遗传风险因素的重要性。目前尚无有效的药物治疗方法可延缓疾病进展，但可能需要对胆管狭窄进行内镜治疗。患者的随访包括对大多数病例中存在的炎症性肠病的管理，以及旨在早期发现胆管癌和结直肠癌的检查，这些癌症的发生频率也较高。在本综述中，我们旨在总结 PSC 的现有知识，特别强调疾病变异性的可能基础。
