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P168. Primary sclerosing cholangitis in the Swiss IBD cohort study: prevalence, patient characteristics, and disease course

M. Fraga1, N. Fournier2, E. Safroneeva3, S. Vavricka4, D. Moradpour1, A. Schoepfer1, 1University Hospital Lausanne / CHUV, Gastroenterology and Hepatology, Lausanne, Switzerland, 2University of Lausanne, Institute of Social and Preventive Medicine, Lausanne, Switzerland, 3University of Bern, Institute of Social and Preventive Medicine, Bern, Switzerland, 4University Hospital Zurich, Gastroenterology and Hepatology, Zurich, Switzerland

Background

Primary sclerosing cholangitis (PSC) represents the most common hepatobiliary extraintestinal manifestation (EIM) in inflammatory bowel disease (IBD). We aimed to assess the prevalence of PSC in the Swiss IBD Cohort Study (SIBDCS) and to identify associated risk factors.

Methods

Patients were enrolled retrospectively (diagnosis before 2006) and prospectively (diagnosis in 2006 and later) into the SIBDCS. Eighty percent of patients were recruited in hospital clinics and 20% in private practice. Patients with IBD and PSC were compared to patients with IBD but without PSC. Non parametric data are presented as median and interquartile range [IQR].

Results

Among 2,187 patients with IBD (1,251 Crohn's disease, CD; 893 ulcerative colitis, UC; 43 indeterminate colitis, IC), diagnosed between 1955 and 2010, 35 patients with PSC were identified (29 PSC-UC, 6 PSC-CD). The cumulative PSC prevalence was 3.2% in UC and 0.5% in CD (p < 0.001 for PSC-UC vs. PSC-CD). PSC was significantly more prevalent in males as compared to females (risk ratio 2.82, p = 0.006). UC-PSC patients had a median age at IBD disease onset of 23 [19–35] years, the median disease duration was 11 [4–17] years, and disease location was in 10.7% proctitis, 21.4% left sided colitis, 10.7% extensive colitis, and in 57.1% pancolitis. Mean age at PSC diagnosis was 30 years in males and 38 years in females. When comparing UC-PSC patients (n = 29) with UC patients without PSC (n = 864) we observed the following correlations: UC-PSC patients were more frequently male (79.3% vs. 54.2%, p = 0.007), and they were younger at UC diagnosis (median 23 [19–35] vs. 31 [24–41] years, p = 0.005). Pancolitis was found in 57.1% of UC-PSC patients compared to 40.6% of UC patients without PSC (p = 0.117). PSC was diagnosed in 71.4% after IBD, in 14.3% at the same time, and in 10.7% before IBD diagnosis (3.6% missing information). Four patients (11.4%) developed cholangiocarcinoma. Four patients were liver transplanted due to recurrent episodes of cholangitis and/or liver failure.

Conclusion

The PSC prevalence in the SIBDCS is comparable to other cohorts from the Unites States and Europe. We identified male gender and young age at IBD diagnosis as risk factors for PSC in UC patients.