| Literature DB >> 29883229 |
James H Tabibian1, Todd H Baron2.
Abstract
INTRODUCTION: 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.Entities:
Keywords: Dominant stricture; balloon dilation; biliary tract disease; cholangioscopy; endoscopy therapy; liver transplantation
Mesh:
Year: 2018 PMID: 29883229 DOI: 10.1080/17474124.2018.1483719
Source DB: PubMed Journal: Expert Rev Gastroenterol Hepatol ISSN: 1747-4124 Impact factor: 3.869