Primary sclerosing cholangitis in patients with inflammatory bowel disease

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of unknown aetiology affecting the biliary tree. It is characterised by inflammation and fibrosisofthe bile duct. In many patients it progresses to biliary cirrhosis and hepatic failure, necessitating liver transplantation. A unique feature of PSC is its strong association with inflammatory bowel disease (IBD). In fact, at least 80% of patients with PSC also suffer from IBD, most commonly ulcerative colitis (UC). Patients with PSC with IBD appeartohave a higher prevalence of mild pancolitis with rectal sparing and with frequent back-wash ileitis. Conversely, only around 2-7% of patients with IBD will develop PSC. Patients with PSC have a high risk of developing of cholangiocarcinoma and life-threatening cholangitis. Liver transplantation remains the only effective option for treatment. This article aims to document two case studies of PSC diagnosis in young patients with ulcerative colitis. In the first case, diagnosis was easy once PSC is considered. The patient additionally had a rare variant of injury to the pancreatic duct. In the second case, PSC diagnosis was more difficult due to the negative cholangiogram. The patient suffered from PSC of the small bile ducts.