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


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


1. Sokol H, Cosnes J, Chazouilleres O et al. Disease activity and cancer risk in inflammatory bowel disease associated with primary sclerosing cholangitis. World J Gastro­enterol 2008; 14(22): 3497–3503.

2. Drastich P, Kamenář D, Wohl P et al. Ulcerative colitis in patiens with primary sclerosing cholangitis (PSC-UC)- a specific subgroup. Čes a Slov Gastroent a Hepatol 2007; 61 (Suppl 1).

3. Loftus EV Jr., Harewood GC, Loftus C. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 2005; 54(1): 91–96.

4. Olsson R, Danielsson A, Jarnerot G et al. Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis. Gastro­enterology 1991; 100 (5 Pt 1): 1319–1323.

5. Broome U, Olsson R, Loof L et al. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut 1996; 38(4): 610–615.

6. Soetikno RM, Lin OS, Heidenreich PA et al. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis: a meta-analysis. Gastrointest Endosc 2002; 56(1): 48–54.

7. Lepistö A, Kärkkäin P, Järvinen HJ et al. Prevalence of primary sclerosing cholangitis in ulcerative colitis patients under­going proctocolectomy and ileal pouch-anal anastomosis. Inflamm Bowel Dis 2008; 14(6): 775–779.

8. Drastich P, Trunečka P, Špičák J. Ulcerative colitis after orthotopic liver transplantation for primary sclerosing cholangitis-a single center experience. Gut 2008; 57 (Suppl II).

9. Tung, Bruce Y Kowdley, Kris V. Epide­miology and pathogenesis of primary sclerosing cholangitis. UpToDate. Accessed: September 2009.

10. Marelli L, Xirouchakis E, Kalambokis G et al. Does the severity of primary sclerosing cholangitis influence the clinical course of associated ulcerative colitis? Gut 2011; 60(9): 1224–1228.

11. Rust C, Brand S. PSC: Protect and Serve with Colitis: does it help the liver to have severe ulcerative colitis? Gut 2011; 60(9): 1165–1166.

12. Jørgensen KK, Grzyb K, Lundin KE e al. Inflammatory bowel disease in patients with primary sclerosing cholangitis: Clinical characterization in liver transplanted and nontransplanted patients. Inflamm Bowel Dis 2011; 18(3): 536–545.

13. Eaton JE et al. High-dose ursodeoxycholic acid is associated with the develop­ment of colorectal neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Am J Gastroenterol 2011; 106(9): 1638–1645.

Paediatric gastroenterology Gastroenterology and hepatology Surgery

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