摘要
原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)是一种慢性胆汁淤积性自身免疫性肝病(autoimmune liver disease,AILD),以肝内胆管和/或肝外胆管的弥漫性炎症、同心性纤维化、局灶性或节段性狭窄为主要特征,最终发展至肝硬化或肝衰竭.PSC的发病机制可能涉及遗传易感性、先天性免疫、获得性免疫及EB病毒感染等.内镜逆行胆管造影、磁共振胰胆管成像、高分辨三维扰相梯度回波序列等技术已用于PSC的影像诊断.此外,胆汁和尿液的蛋白质组学分析可能有助于PSC并发胆管癌的早期诊断.熊去氧胆酸(ursodeoxycholic acid,UDCA)、内镜以及肝移植是治疗PSC的方法.上述最新进展为PSC的诊断和治疗提供了新的思路.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic autoimmune liver disease character- ized by diffuse in? ammation, concentric f ibrosis, focal or segmental stricture of intrahepatic and/or extrahepatic bile ducts, which can eventually lead to cirrhosis or hepatic function failure. The patho- genesis of PSC may involve genetic susceptibility, innate or adaptive immunity, and Epstein-Barr virus infection. Diagnostic imaging modalities include endoscopic retrograde cholangiography, magnetic resonance cholangiopancreatography, and high-resolution three-dimensional SPGR. Proteomic analysis of bile and urine may become a new tool for early diagnosis of cholangiocar- cinoma in PSC patients. Ursodeoxycholic acid (UDCA), endoscopic therapy and liver transplan- tation are major treatments for PSC.
出处
《世界华人消化杂志》
CAS
北大核心
2012年第31期2978-2985,共8页
World Chinese Journal of Digestology
关键词
原发性硬化性胆管炎
发病机制
诊断
治疗
Primary sclerosing cholangitis
Patho- genesis
Diagnosis
Therapy