Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study

Basavaraj Kerur, Eric I. Benchimol, Karoline Fiedler, Marisa Stahl, Jeffrey Hyams, Michael Stephens, Ying Lu, Marian Pfefferkorn, Raza Alkhouri, Jennifer Strople, Judith Kelsen, Leah Siebold, Alka Goyal, Joel R. Rosh, Neal Leleiko, Johan Van Limbergen, Anthony L. Guerrerio, Ross Maltz, Lina Karam, Eileen CrowleyAnne Griffiths, Melvin B. Heyman, Mark Deneau, Keith Benkov, Joshua Noe, Dedrick Mouton, Helen Pappa, Joseph A. Galanko, Scott Snapper, Aleixo M. Muise, Michael D. Kappelman

Research output: Contribution to journalArticlepeer-review

Abstract

Background: The incidence of very early onset inflammatory bowel disease (VEOIBD) is increasing, yet the phenotype and natural history of VEOIBD are not well described. Methods: We performed a retrospective cohort study of patients diagnosed with VEOIBD (6 years of age and younger) between 2008 and 2013 at 25 North American centers. Eligible patients at each center were randomly selected for chart review. We abstracted data at diagnosis and at 1, 3, and 5 years after diagnosis. We compared the clinical features and outcomes with VEOIBD diagnosed younger than 3 years of age with children diagnosed with VEOIBD at age 3 to 6 years. Results: The study population included 269 children (105 [39%] Crohn's disease, 106 [39%] ulcerative colitis, and 58 [22%] IBD unclassified). The median age of diagnosis was 4.2 years (interquartile range 2.9-5.2). Most (94%) Crohn's disease patients had inflammatory disease behavior (B1). Isolated colitis (L2) was the most common disease location (70% of children diagnosed younger than 3 years vs 43% of children diagnosed 3 years and older; P = 0.10). By the end of follow-up, stricturing/penetrating occurred in 7 (6.6%) children. The risk of any bowel surgery in Crohn's disease was 3% by 1 year, 12% by 3 years, and 15% by 5 years and did not differ by age at diagnosis. Most ulcerative colitis patients had pancolitis (57% of children diagnosed younger than 3 years vs 45% of children diagnosed 3 years and older; P = 0.18). The risk of colectomy in ulcerative colitis/IBD unclassified was 0% by 1 year, 3% by 3 years, and 14% by 5 years and did not differ by age of diagnosis. Conclusions: Very early onset inflammatory bowel disease has a distinct phenotype with predominantly colonic involvement and infrequent stricturing/penetrating disease. The cumulative risk of bowel surgery in children with VEOIBD was approximately 14%-15% by 5 years. These data can be used to provide anticipatory guidance in this emerging patient population.

Original languageEnglish (US)
Pages (from-to)295-302
Number of pages8
JournalInflammatory bowel diseases
Volume27
Issue number3
DOIs
StatePublished - Mar 1 2021

Keywords

  • VEOIBD
  • epidemiology
  • surgery

ASJC Scopus subject areas

  • Immunology and Allergy
  • Gastroenterology

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