• Montserrat Fraga, Nicolas Fournier, Ekaterina Safroneeva, Valérie Pittet, Sébastien Godat, Alex Straumann, Andreas Nydegger, Stephan R Vavricka, Darius Moradpour, Alain M Schoepfer, and Swiss IBD Cohort Study Group.
• aDepartment of Internal Medicine, Division of Gastroenterology and Hepatology bInstitute of Social and Preventive Medicine cDepartment of Pediatrics, Division of Pediatric Gastroenterology and Hepatology, University Hospital Lausanne and University of, Lausanne dInstitute of Social and Preventive Medicine, University of Bern, Bern eDepartment of Internal Medicine, Division of Gastroenterology and Hepatology, University Hospital Basel, Basel fDepartment of Internal Medicine, Division of Gastroenterology and Hepatology, Stadtspital Triemli gDepartment of Internal Medicine, Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland.
• Eur J Gastroenterol Hepatol. 2017 Jan 1; 29 (1): 91-97.

Background And AimPrimary sclerosing cholangitis (PSC) represents the most common hepatobiliary extraintestinal manifestation of inflammatory bowel disease (IBD). We aimed to assess the prevalence of PSC in the Swiss Inflammatory Bowel Disease Cohort Study, to identify associated risk factors, and to describe the long-term evolution.Patients And MethodsData of patients enrolled into the Swiss Inflammatory Bowel Disease Cohort Study were analyzed. Logistic regression modeling was performed to identify risk factors for PSC.ResultsAmong 2744 patients [1188 ulcerative colitis (UC); 1556 Crohn's disease (CD)], 57 had PSC (48 UC-PSC, nine CD-PSC). The prevalence of PSC was higher in UC compared with CD (4.04 vs. 0.58%, P<0.001). We identified the following significant independent risk factors for PSC in patients with UC: male sex [odds ratio (OR) 2.771, P=0.022], pancolitis (OR 2.855, P=0.011), nonsmoker at diagnosis (OR 9.253, P=0.030), and a history of appendicectomy (OR 4.114, P=0.019). During a median follow-up time of 74.8 months, four (7.0%) of PSC patients developed cholangiocarcinoma, six (10.5%) underwent liver transplantation, and five (8.8%) died. Survival of IBD-PSC patients was significantly worse compared with IBD patients without PSC (P=0.001). UC-PSC patients developed significantly more frequently colorectal cancer compared with UC patients without PSC (2/48 vs. 9/1440, P=0.017).ConclusionApproximately 4% of UC patients and 0.6% of CD patients had PSC. Male sex, pancolitis, nonsmoker status, and a history of appendicectomy were significantly associated with PSC. PSC is associated with considerable morbidity and mortality in the long term.

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