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Sclerosing cholangitis

机译：硬化性胆管炎

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Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disorder characterized by strictures and dilatations of the biliary tree. The median survival has been reported to be 12 years from diagnosis but patients diagnosed during the last decade have been shown to have longer survival of a median of 18 years. Small duct PSC seems to be an entity of its own and rarely progresses to large duct PSC and the prognosis of small duct PSC is much better than in large duct disease. Cholangiocarcinoma does not develop in patients with small duct PSC according to recently published studies. Autoantibodies binding to biliary epithelial cells have been demonstrated in sera from patients with PSC but the pathogenic role of these remains to be determined. It is suggested that inflammatory bowel disease is accelerated after orthotopic liver transplantation. MR cholangiopancreatography is increasingly used in clinical practice and has been reported to have similar diagnostic accuracy as ERCP. However, lack of standardization of this novel technology makes it unclear if it can supersede ERCP as a diagnostic tool in PSC at the present time. Better diagnostic tests to detect Cholangiocarcinoma in patients with PSC are urgently needed.
机译：原发性硬化性胆管炎（PSC）是一种慢性胆汁淤积性肝病，其特征是胆管狭窄和扩张。据报道，中位生存期为诊断后的12年，但最近十年被诊断出的患者的中位生存期为18年。小导管PSC似乎是其自身的一个实体，很少进展为大导管PSC，而小导管PSC的预后要好于大导管疾病。根据最近发表的研究，小导管PSC患者不发生胆管癌。在PSC患者的血清中已经证明了与胆管上皮细胞结合的自身抗体，但这些致病作用尚待确定。建议原位肝移植后炎症性肠病加速。 MR胰胆管造影术在临床实践中越来越多，据报道其诊断准确性与ERCP相似。但是，由于缺乏对这一新颖技术的标准化，目前尚不清楚它是否可以取代ERCP作为PSC中的诊断工具。迫切需要更好的诊断测试来检测PSC患者的胆管癌。

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