De Vries Boudewijn, Weersma Rinse K
Department of Gastroenterology and Hepatology, University of Groningen and University Medical Center Groningen, Groningen, the Netherlands -
Minerva Gastroenterol Dietol. 2016 Mar;62(1):49-62. Epub 2015 Oct 7.
Primary sclerosing cholangitis (PSC) is a rare chronic liver disease of unknown etiology for which the only known curative treatment is liver transplantation. The disease is defined by progressive inflammation and fibrosis of the bile ducts, causing biliary strictures and cholestasis. Common complications of the disease are the presence of biliary lithiasis requiring stone extraction, and development of dominant bile duct strictures requiring balloon dilatation and stent placement through endoscopic retrograde cholangiopancreatography. The increased development of cholangiocarcinoma is a dreaded complication in PSC, as it is often detected in an advanced stage and is associated with a poor prognosis. Several endoscopic techniques, including endoscopic ultrasound, confocal laser endomicroscopy and peroral cholangioscopy are applied in the management of PSC and detection of cholangiocarcinoma. Tissue sampling through different types of biopsies and biliary brush combined with fluorescence in situ hybridization are used to differentiate benign dominant strictures from biliary neoplasia. Nonetheless early detection of cholangiocarcinoma in PSC remains a clinical challenge requiring a specialized diagnostic workup. The aim of this review is to discuss the role of diagnostic and therapeutic endoscopy in management of PSC, providing an overview of current literature.
原发性硬化性胆管炎(PSC)是一种病因不明的罕见慢性肝病,目前已知唯一的治愈性治疗方法是肝移植。该疾病的定义是胆管进行性炎症和纤维化,导致胆管狭窄和胆汁淤积。该疾病的常见并发症包括需要取石的胆石症,以及需要通过内镜逆行胰胆管造影进行球囊扩张和支架置入的主要胆管狭窄。胆管癌发病率增加是PSC中令人恐惧的并发症,因为它通常在晚期被发现,且预后较差。包括内镜超声、共聚焦激光显微内镜和经口胆管镜在内的几种内镜技术被应用于PSC的管理和胆管癌的检测。通过不同类型的活检和胆管刷检结合荧光原位杂交进行组织采样,用于区分良性主要狭窄和胆管肿瘤。尽管如此,PSC中胆管癌的早期检测仍然是一项临床挑战,需要专门的诊断检查。本综述的目的是讨论诊断性和治疗性内镜在PSC管理中的作用,概述当前的文献。