title: Extraintestinal Manifestations of Pediatric Inflammatory Bowel Disease: Prevalence, Presentation, and Anti-TNF Treatment.
abstract: There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD).
  
  
  
  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.
  
  
  
  A 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%).
  
  
  
  In 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.
authors: *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.
authors: Thomas
authors: T
authors: Greuter
authors: Fabio
authors: F
authors: Bertoldo
authors: Roman
authors: R
authors: Rechner
authors: Alex
authors: A
authors: Straumann
authors: Luc
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authors: Biedermann
authors: Jonas
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authors: Zeitz
authors: Benjamin
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authors: Misselwitz
authors: Michael
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authors: Scharl
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authors: Rogler
authors: Ekaterina
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authors: Safroneeva
authors: Raja A R
authors: RAR
authors: Ali
authors: Christian
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authors: Braegger
authors: Klaas
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authors: Heyland
authors: Pascal
authors: P
authors: Mueller
authors: Andreas
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authors: Nydegger
authors: Laetitia-Marie
authors: LM
authors: Petit
authors: Susanne
authors: S
authors: Schibli
authors: Raoul I
authors: RI
authors: Furlano
authors: Johannes
authors: J
authors: Spalinger
authors: Michela
authors: M
authors: Schäppi
authors: Samuel
authors: S
authors: Zamora
authors: Florian
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authors: Froehlich
authors: Denise
authors: D
authors: Herzog
authors: Alain M
authors: AM
authors: Schoepfer
authors: Stephan R
authors: SR
authors: Vavricka
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*; D003093:Colitis, Ulcerative / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D003424:Crohn Disease / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007592:Joint Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*; D016015:Logistic Models; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D012871:Skin Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors; D014605:Uveitis / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*
chemical_list: D000893:Anti-Inflammatory Agents; D014409:Tumor Necrosis Factor-alpha