• Thomas Greuter, Fabio Bertoldo, Roman Rechner, Alex Straumann, Luc Biedermann, Jonas Zeitz, Benjamin Misselwitz, Michael Scharl, Gerhard Rogler, Ekaterina Safroneeva, Raja A R Ali, Christian Braegger, Klaas Heyland, Pascal Mueller, Andreas Nydegger, Laetitia-Marie Petit, Susanne Schibli, Raoul I Furlano, Johannes Spalinger, Michela Schäppi, Samuel Zamora, Florian Froehlich, Denise Herzog, Alain M Schoepfer, Stephan R Vavricka, and Swiss IBD Cohort Study Group.
• *Division of Gastroenterology and Hepatology, University Hospital Zurich †Division of Gastroenterology and Hepatology, Triemli Hospital Zurich, Zurich ‡Praxis Römerhof, Olten §Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland ||Division of Gastroenterology, The National University of Malaysia Medical Centre, Kuala Lumpur, Malaysia ¶Division of Gastroenterology and Nutrition and Children's Research Center, University Children's Hospital Zurich, Zurich #Department of Pediatrics, Kantonsspital Winterthur, Winterthur **Division of Gastroenterology and Hepatology, Kinderspital St Gallen, St Gallen ††Division of Pediatric Gastroenterology and Hepatology, Department of Pediatrics, University Hospital Lausanne-CHUV, Lausanne ‡‡Department of Pediatrics, University Hospital Geneva-HCUG, Geneva §§Division of Gastroenterology and Hepatology, University Children's Hospital Bern, Bern ||||Division of Gastroenterology and Nutrition, University Children's Hospital Basel-UKBB, Basel ¶¶Department of Pediatrics, Kantonsspital Luzern-LUKS, Luzern ##Private Practice, Pediatric Unit, Clinique des Grangettes, Geneva ***Private Practice, Onex †††Division of Gastroenterology and Hepatology, University Hospital Basel, Basel ‡‡‡Division of Gastroenterology and Hepatology, University Hospital Lausanne-CHUV, Lausanne §§§Department of Pediatrics, Hopital Cantonal de Fribourg, Fribourg, Switzerland.
• J. Pediatr. Gastroenterol. Nutr. 2017 Aug 1; 65 (2): 200-206.

BackgroundThere is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD).MethodsSince 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively.ResultsA total of 55 patients (16.7%) experienced 1-4 EIM (39 Crohn disease, 12 ulcerative colitis, and 4 IBD-unclassified 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 Crohn disease when compared to ulcerative colitis/IBD-unclassified (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%). Approximately 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). Thirty-one of the 55 patients (56.4%) were treated with 1 or more anti-tumor necrosis factor (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%).ConclusionsIn a cohort of pediatric patients with IBD, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings may 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 may present despite ongoing anti-TNF therapy.

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