Infantile and Very Early Onset Inflammatory Bowel Disease: A Multicenter Study

Pediatrics. 2024 Aug 1;154(2):e2023064546. doi: 10.1542/peds.2023-064546.

Abstract

Objectives: This study described disease characteristics and long-term outcomes in patients diagnosed with very early onset inflammatory bowel disease (VEOIBD) (diagnosed before 6 years of age) and infantile-IBD (before 2 years).

Methods: Cases from 21 centers worldwide diagnosed with VEOIBD (2008-2018), with minimum 2 years of follow-up, were retrospectively reviewed.

Results: The cohort included 243 patients (52% males, median follow-up of 5.8 [range 2-18] years, including 69 [28%]) with infantile-IBD. IBD subtypes included Crohn's disease (CD), ulcerative colitis (UC), or IBD-unclassified (IBDU) in 30%, 59%, and 11%, respectively. Among patients with CD, 94% had colonic involvement, and among patients with UC/IBDU, 75% had pancolitis. Patients with infantile-IBD presented with higher rates of IBDU, lower hemoglobin and albumin levels, and higher C-reactive protein, and had lower response rates to first-induction therapy and corticosteroids therapy (P < .05 for all). Colectomy and diversion surgeries were performed in 11% and 4%, respectively, with no significant differences between age groups. Corticosteroid-free remission rates were 74% and 78% after 3 and 5 years, respectively, and 86% at end of follow-up. Genetic testing was performed in 96 (40%) patients. Among tested population, 15 (16%) were identified with monogenic disease. This group demonstrated lower response rates to induction therapies, higher rates of surgical intervention, and higher rates of major infections (P < .05 for all).

Conclusions: Patients with VEOIBD, including infantile-IBD, exhibit low rate of complications and surgical interventions at the long term. Patients with monogenic IBD are at risk for more severe disease course.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Age of Onset*
  • Child
  • Child, Preschool
  • Colitis, Ulcerative / diagnosis
  • Colitis, Ulcerative / epidemiology
  • Colitis, Ulcerative / genetics
  • Colitis, Ulcerative / surgery
  • Colitis, Ulcerative / therapy
  • Crohn Disease / diagnosis
  • Crohn Disease / epidemiology
  • Crohn Disease / genetics
  • Crohn Disease / surgery
  • Crohn Disease / therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Inflammatory Bowel Diseases / diagnosis
  • Inflammatory Bowel Diseases / epidemiology
  • Inflammatory Bowel Diseases / genetics
  • Inflammatory Bowel Diseases / therapy
  • Male
  • Retrospective Studies