Prognosis of pediatric-onset inflammatory bowel disease associated with primary sclerosing cholangitis: A population-based study

Abstract

Objectives: To assess whether the prognosis of pediatric-onset inflammatory bowel disease (IBD) is influenced by its association with primary sclerosing cholangitis (PSC) considering medical treatment, bowel resection, risk of cancer, and mortality.

Methods: A retrospective population-based study was conducted using data from the EPIMAD Registry, one of the most extensive prospective population-based IBD studies globally. Cases (IBD-PSC) were compared with matched controls (matched-IBD). Inclusion criteria were age ≤17 years at IBD diagnosis and follow-up ≥2 years. PSC was confirmed by magnetic resonance cholangiopancreatography and/or histology. Each case was matched to four controls by propensity score (i.e., sex, age, year, and location of IBD at diagnosis).

Results: We included 24 cases and 96 controls. Median duration of follow-up was 6.4 years (interquartile range = 3.1-14.3). No significant difference was observed between the two groups in terms of exposure to treatment at 5 years (immunosuppressants, corticosteroids, or antitumor necrosis factor). In IBD-PSC, cancers were 28 times more frequent (standardized incidence ratio = 27.9; 95% confidence interval [CI], 7.0-111.7, p = 0.002), and death was 13 times more frequent (standardized mortality ratio = 13.3; 95% CI, 3.3-53.4, p = 0.010) than in the general population. No increased risk of cancer or mortality was observed in patients with IBD but without PSC compared to the general population.

Conclusion: Although the course of IBD is not different, the prognosis of pediatric-onset IBD associated with PSC is significantly worse than that of pediatric-onset IBD without PSC because of increased cancer and mortality rates.

Keywords: Crohn's disease; cancer; children; liver disease; mortality; treatment; ulcerative colitis.