Primary Sclerosing Cholangitis in Children With Inflammatory Bowel Diseases is Associated with Milder Clinical Activity but More Frequent Subclinical Inflammation and Growth Impairment

Abstract Background & Aims Although inflammatory bowel diseases (IBD) associated with primary sclerosing cholangitis (PSC) have been well characterized in adults, there have been few pediatric studies, and these were small and produced conflicting results. We investigated features of PSC-IBD in children, compared to children with IBD without PSC. Methods We performed a retrospective study of 74 children with PSC-IBD, diagnosed from 2000 through 2018, who were each matched with 2 children with ulcerative colitis or IBD-unclassified (controls) based on sex, date of birth, and type of IBD. We compared IBD distribution and clinical activity (remission, medication use, hospitalization, or colectomy) and patient growth between groups. Data were extracted from each hospital contact and analyzed using mixed effects analyses or Cox proportional hazards regression, adjusting for time-dependent medication exposure. Results Higher proportions of children with PSC-IBD had backwash ileitis, pancolitis, and rectal sparing, and more severe right-sided disease, than controls (P Conclusions In a retrospective study, we found that features of IBD differed between children with vs without PSC, similar to adults. Despite the mild clinical activity of IBD in patients with PSC, lack of symptoms does not always indicate lack of mucosal inflammation. Children with PSC-IBD have greater growth impairments compared to children with ulcerative colitis or IBD-unclassified.