Primary sclerosing cholangitis in patients with inflammatory bowel disease


Authors: P. Matějková 1;  P. Drastich 2;  A. Jungwirthová 1
Authors‘ workplace: Sanatorium sv. Anny, EGK s. r. o., Praha, 2Klinika hepatogastroenterologie, IKEM, Praha 1
Published in: Gastroent Hepatol 2012; 66(3): 210-213
Category: IBD: Case Report

Overview

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of unknown aetiology affecting the biliary tree. It is characterised by inflammation and fibrosis of the 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 appear to have 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.

Key words:
inflammatory bowel disease – primary sclerosing cholangitis – liver tests


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Labels
Paediatric gastroenterology Gastroenterology and hepatology Surgery

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Gastroenterology and Hepatology

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2012 Issue 3

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