Background: Primary sclerosing cholangitis (PSC) has been shown to increase the risk for chronic pouchitis. However, the association between PSC and Crohn's disease (CD) of the pouch has not been studied.
Methods: Consecutive inflammatory bowel disease patients undergoing proctocolectomy with ileal pouch-anal anastomosis in our Pouchitis Registry from 2002 to 2012 were studied. Cases consisted of patients with CD of the pouch. Both univariable and multivariable analyses were performed.
Results: A total of 1425 patients met the inclusion criteria, including 265 (18.6%) with CD of the pouch and 78 (5.5%) with PSC. In the whole cohort, 799 patients (56.1%) were male and the mean ages at the time of diagnosis of inflammatory bowel disease and at pouch surgery were 25.5 ± 12.3 years and 35.4 ± 13.9 years, respectively. Patients with PSC had a longer duration from inflammatory bowel disease diagnosis to pouch construction (P < 0.001). Fewer patients with PSC had toxic megacolon at the time of colectomy (P = 0.009), but more patients with PSC had neoplasia as the indication for colectomy (P < 0.001), concurrent autoimmune disorders (P < 0.001), and liver transplantation (P = 0.001). In the multivariate analysis, the presence of PSC was shown to be inversely associated with the risk for the development of CD of the pouch, with a hazard ratio of 0.39 (95% confidence interval: 0.16 to 0.95, P = 0.038). However, no significant difference in terms of the distribution of phenotypes of CD of the pouch between patients with and without PSC was identified (P = 0.59).
Conclusions: The presence of PSC is inversely associated with the development of CD of the pouch.