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

• Published in: Pediatrics (Evanston) 2024-08, Vol.154 (2), p.1
• Main Authors: Guz-Mark, Anat, Aloi, Marina, Scarallo, Luca, Bramuzzo, Matteo, Escher, Johanna C, Alvisi, Patrizia, Henderson, Paul, Hojsak, Iva, Lev-Tzion, Raffi, El-Matary, Wael, Schwerd, Tobias, Granot, Maya, Sladek, Malgorzata, Strisciuglio, Caterina, Müller, Katalin E, Olbjørn, Christine, Tzivinikos, Christos, Yerushalmy-Feler, Anat, Huysentruyt, Koen, Norsa, Lorenzo, Viola, Irene, de Ridder, Lissy, Shouval, Dror S, Lega, Sara, Lionetti, Paolo, Catassi, Giulia, Assa, Amit
• Format: Article
• Language: English
• Subjects: Adolescent; Age of Onset; C-reactive protein; Child; Child, Preschool; Colitis, Ulcerative - diagnosis; Colitis, Ulcerative - epidemiology; Colitis, Ulcerative - genetics; Colitis, Ulcerative - surgery; Colitis, Ulcerative - therapy; Corticosteroids; Crohn Disease - diagnosis; Crohn Disease - epidemiology; Crohn Disease - genetics; Crohn Disease - surgery; Crohn Disease - therapy; Crohn's disease; Female; Follow-Up Studies; Hemoglobin; Humans; Induction therapy; Infant; Inflammatory bowel disease; Inflammatory bowel diseases; Inflammatory Bowel Diseases - diagnosis; Inflammatory Bowel Diseases - epidemiology; Inflammatory Bowel Diseases - genetics; Inflammatory Bowel Diseases - therapy; Intestine; Male; Population genetics; Remission; Response rates; Retrospective Studies; Steroids; Surgery; Ulcerative colitis
• ISSN: 0031-4005; 1098-4275; 1098-4275
• DOI: 10.1542/peds.2023-064546

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