MacFaul George R, Chapman Roger W
Department of Gastroenterology, John Radcliffe Hospital, Oxford, UK.
Curr Opin Gastroenterol. 2006 May;22(3):288-93. doi: 10.1097/01.mog.0000218966.78558.67.
Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree complicated by cirrhosis and cholangiocarcinoma. It is immune mediated although the precise etiology remains unknown.
Research into etiopathogenesis, epidemiology, the relationship with inflammatory bowel disease, diagnosis of cholangiocarcinoma, medical therapy, and the outcome of liver transplantation are discussed.
It is likely that a number of patients previously diagnosed with primary sclerosing cholangitis have autoimmune pancreatitis in association with primary sclerosing cholangitis, a syndrome with distinct clinicopathological features including steroid responsiveness. Primary sclerosing cholangitis-inflammatory bowel disease probably represents a distinct inflammatory bowel disease phenotype, which has implications for colonoscopic surveillance of these patients. CA19-9 plays no surveillance role for the early detection of cholangiocarcinoma. The best-studied drug in primary sclerosing cholangitis is ursodeoxycholic acid, which, despite a range of potentially valuable actions on the cholestatic liver, has not yet been proved to make a substantial impression on the course of the disease. Orthotopic liver transplantation remains the only established long-term treatment for primary sclerosing cholangitis.
原发性硬化性胆管炎是一种慢性胆汁淤积性肝病，其特征为胆管树狭窄，并伴有肝硬化和胆管癌。尽管确切病因尚不清楚，但它是由免疫介导的。
讨论了原发性硬化性胆管炎的病因发病机制、流行病学、与炎症性肠病的关系、胆管癌的诊断、药物治疗以及肝移植的预后。
许多先前被诊断为原发性硬化性胆管炎的患者可能同时患有自身免疫性胰腺炎和原发性硬化性胆管炎，这是一种具有独特临床病理特征（包括对类固醇有反应）的综合征。原发性硬化性胆管炎 - 炎症性肠病可能代表一种独特的炎症性肠病表型，这对这些患者的结肠镜监测具有重要意义。CA19 - 9在胆管癌的早期检测中不具有监测作用。在原发性硬化性胆管炎中研究最多的药物是熊去氧胆酸，尽管它对胆汁淤积性肝脏有一系列潜在的有益作用，但尚未被证明能对疾病进程产生实质性影响。原位肝移植仍然是原发性硬化性胆管炎唯一已确立的长期治疗方法。
