Inflammatory Bowel Disease Phenotype in Pediatric Primary Sclerosing Cholangitis

Abstract

Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that most often occurs in association with inflammatory bowel disease (IBD). We examined whether the activity or colonic distribution of IBD differed in pediatric patients with and without PSC. Methods: We compared colonic disease distribution, physician global assessment scores, Mayo endoscopic severity scores, IBD-related hospital admissions, and colonic resection surgery rate in a retrospective cohort of pediatric patients with IBD with and without PSC. Results: We identified 37 patients with PSC-IBD, and 137 non-PSC matched IBD controls. Pancolitis was seen in 89.7 versus 72.4% (P 0.051) of patients with PSC-IBD and rectal sparing in 24.3 versus 21.6% (P 0.721) of patients with IBD. Physician global assessment and Mayo scores at presentation and in follow-up were similar in PSC-IBD and IBD. Patients with PSC-IBD had 0.19 admissions per person-year compared with 0.25 in patients with IBD. The incidence rate ratio for admission was 0.75 (95% confidence interval (CI), 0.51-1.08). The 5-year probability of colonic surgery was 16.4% (95% CI, 7.0-36.0) in patients with PSC-IBD and 24.7% (95% CI, 17.7-33.8) in patients with IBD (P 0.271). In a multivariate model, male sex (hazard ratio [HR] 2.2 [95% CI, 1.1-4.3]) and the presence of a non-PSC immune-mediated comorbidity {HR 3.9 (95% CI, 1.5-10.4), but not PSC (HR 0.5 [95% CI, 0.2-1.3])} or Crohn's disease (HR 0.5 [95% CI, 0.1-1.5]), were risk factors for colonic surgery in pediatric IBD. Conclusions: Patients with IBD and PSC were more likely to present with pancolitis, but had similar rates of rectal sparing. Patients with IBD showed similar disease activity across a wide range of measures, at presentation and in follow-up, regardless of the presence of PSC.