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

J Pediatr Gastroenterol Nutr. 2017 Aug;65(2):200-206. doi: 10.1097/MPG.0000000000001455.

Abstract

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: 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%).

Conclusions: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Anti-Inflammatory Agents / therapeutic use
  • Child
  • Child, Preschool
  • Cholangitis, Sclerosing / diagnosis
  • Cholangitis, Sclerosing / drug therapy
  • Cholangitis, Sclerosing / epidemiology
  • Cholangitis, Sclerosing / etiology*
  • Colitis, Ulcerative / complications*
  • Colitis, Ulcerative / diagnosis
  • Colitis, Ulcerative / drug therapy
  • Crohn Disease / complications*
  • Crohn Disease / diagnosis
  • Crohn Disease / drug therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Infant, Newborn
  • Joint Diseases / diagnosis
  • Joint Diseases / drug therapy
  • Joint Diseases / epidemiology
  • Joint Diseases / etiology*
  • Logistic Models
  • Male
  • Prevalence
  • Retrospective Studies
  • Skin Diseases / diagnosis
  • Skin Diseases / drug therapy
  • Skin Diseases / epidemiology
  • Skin Diseases / etiology*
  • Tumor Necrosis Factor-alpha / antagonists & inhibitors
  • Uveitis / diagnosis
  • Uveitis / drug therapy
  • Uveitis / epidemiology
  • Uveitis / etiology*

Substances

  • Anti-Inflammatory Agents
  • Tumor Necrosis Factor-alpha

Supplementary concepts

  • Pediatric Crohn's disease
  • Pediatric ulcerative colitis