原发性硬化性胆管炎的临床研究进展

Progress in clinical research of primary sclerosing cholangitis

原发性硬化性胆管炎(PSC)是一种慢性胆汁淤积性自身免疫性肝病,以肝内胆管和/或肝外胆管弥漫性炎症、同心性纤维化、局灶性或节段性狭窄为主要特征,最终导致胆管阻塞、胆汁淤积性肝硬化、肝衰竭。PSC发病机制不明,易发生漏误诊,该病目前尚无有效的治疗药物,故预后不良,现就PSC临床、病理、实验室及影像学检查等方面研究进展做一综述,以期提高对本病认识。

Primary sclerosing cholangitis（PSC） is a chronic autoimmune cholestatic liver disease,with intrahep atic bile duct and / or extrahepatic bile duct diffuse inflammation, concentric fibrosis, focal or segmental stenosis as the main features. Eventually this disease leads to bile duct obstruction, biliary cirrhosis and liver failure. For PSC is not a clinical common disease, and its pathogenesis is unknown, prone to misdiagnosis phenomenon, the disease hasn＇t been ef fectively treated currently, so the prognosis is poor. This paper reviewed the progress in the researches of clinic, pathology,laboratory and imaging examinations of this disease.