Endoscopic management of primary sclerosing cholangitis

Abstract Primary sclerosing cholangitis (PSC) remains a rare but potentially devastating chronic, cholestatic liver disease. PSC causes obstruction of intra- and/or extra-hepatic bile ducts by inflammation and fibrosis, leading to biliary obstruction, cirrhosis and portal hypertension with all associated sequelae. The most dreaded consequence of PSC is cholangiocarcinoma, occurring in 10-20% of patients with PSC, and with population-based estimates of a 398-fold increased risk of cholangiocarcinoma in patients with PSC compared to the general population. We use the 4-D approach to endoscopic evaluation and management of PSC based on currently available evidence. After laboratory testing with liver chemistries and high-quality cross-sectional imaging with MRCP, the first D is D ominant stricture diagnosis and evaluation. Second, D ilation of strictures found during ERCP is performed using balloon dilation to as many segments as possible. Third, D ysplasia and cholangiocarcinoma diagnosis is performed by separated brushings for conventional cytology and fluorescence in situ hybridization (FISH), and consideration for direct cholangioscopy with SpyGlass™. Fourth and finally, D osing of antibiotics is critical to prevent peri-procedural cholangitis. The aim of this review article is to explore endo-scopic tools and techniques for the diagnosis and management of PSC and provide a practical approach for clinicians.