Extraintestinal Manifestations of Pediatric Inflammatory Bowel Disease: Prevalence, Presentation and anti-TNF Treatment.

Background: There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD). Methods: Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively. Results: 55 patients (16.7%) suffered from 1-4 EIM (39 Crohn's disease, 12 ulcerative colitis and 4 IBD-Unclassified (IBD-U) patients). At IBD onset, presence of EIM was more frequent than in the adult population (8.5% vs. 5.0%, p = 0.014). EIM were more frequent in CD when compared to UC/IBD-U (22.5 vs. 10.3%, p = 0.003). The most prevalent EIM were peripheral arthritis (26/329, 7.9%) and aphthous stomatitis (24/329, 7.3%). 27.6% of all EIM appeared before IBD diagnosis. Median time between IBD diagnosis and occurrence of first EIM was 1 month (-37.5-149.0). 31 of the 55 patients (56.4%) were treated with one or more anti-TNF agents. IBD patients with EIM were more likely to be treated with anti-TNF compared to those without (56.4% vs. 35.0%, p = 0.003). Response rates to anti-TNF depended on underlying EIM and were best for peripheral arthritis (61.5%) and uveitis (66.7%). Conclusions: In a cohort of pediatric IBD patients, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings might translate into an increased awareness of underlying IBD, thereby decreasing diagnostic delay. Anti- TNF for the treatment of certain EIM is effective although a substantial proportion of new EIM might present despite ongoing anti-TNF therapy. (C) 2016 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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