a Division of Gastroenterology, Department of Medicine , Olive View-UCLA Medical Center , Sylmar , CA , USA.
b Division of Gastroenterology and Hepatology , University of North Carolina at Chapel Hill , Chapel Hill , NC , USA.
Expert Rev Gastroenterol Hepatol. 2018 Jul;12(7):693-703. doi: 10.1080/17474124.2018.1483719. Epub 2018 Jun 18.
Primary sclerosing cholangitis (PSC) is a rare but clinically important cholestatic liver disease. Histopathologically and cholangiographically, PSC is characterized by intra- and/or extra-hepatic bile duct inflammation and fibro-obliteration, which ultimately leads to biliary cirrhosis and related sequelae, including development of hepatobiliary and colorectal carcinomata. PSC can be diagnosed at essentially any age and carries a median survival of 15-20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma. Given the chronic and progressive nature of PSC, its inherent association with both neoplastic and non-neoplastic biliary tract complications, and the lack of effective pharmacotherapies, alimentary and biliary tract endoscopy plays a major role in the care of patients with PSC. Areas covered: Here, we provide a narrative review on endoscopic management of PSC, including established and evolving applications to the diagnosis and treatment of both its benign and malignant complications. Expert commentary: Due to the rarity of PSC and the considerable patient-years required to rigorously study major endpoints, there remains a paucity of high-quality evidence regarding its management. As the advanced endoscopic repertoire expands, so has the interest in developing best practices in PSC, which we discuss herein.
原发性硬化性胆管炎(PSC)是一种罕见但具有重要临床意义的胆汁淤积性肝病。从组织病理学和胆管造影上看,PSC 的特征为肝内外胆管炎症和纤维性闭塞,最终导致胆汁性肝硬化及相关并发症,包括肝胆和结直肠癌的发生。PSC 可在任何年龄诊断,中位生存时间为 15-20 年,与诊断时的年龄无关,是胆管癌的主要危险因素。鉴于 PSC 的慢性和进行性特征,其与肿瘤性和非肿瘤性胆道并发症的固有相关性,以及缺乏有效的药物治疗,饮食和胆道内镜在 PSC 患者的治疗中发挥着重要作用。涵盖领域:本文就 PSC 的内镜管理进行了叙述性综述,包括其良性和恶性并发症的诊断和治疗中的既定和不断发展的应用。专家评论:由于 PSC 的罕见性以及严格研究主要终点所需的大量患者年数,其管理仍缺乏高质量的证据。随着先进的内镜技术不断发展,人们对制定 PSC 的最佳实践产生了浓厚的兴趣,我们在本文中对此进行了讨论。