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.
