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

Area of Science:

• Pediatric Gastroenterology
• Inflammatory Bowel Disease Research
• Genetics of Gastrointestinal Disorders

Background:

• Inflammatory bowel disease (IBD) diagnosed before 6 years of age (VEOIBD) and before 2 years (infantile-IBD) represents a distinct clinical challenge.
• Understanding the specific characteristics and long-term trajectories of these young patients is crucial for optimizing management.

Purpose of the Study:

• To describe the disease characteristics and evaluate the long-term outcomes of patients diagnosed with VEOIBD and infantile-IBD.
• To identify factors associated with disease severity and treatment response in this pediatric population.

Main Methods:

• Retrospective review of VEOIBD cases diagnosed between 2008-2018 across 21 international centers.
• Inclusion criteria required a minimum of 2 years of follow-up data.

Main Results:

• The cohort comprised 243 patients (52% males), with 69 diagnosed with infantile-IBD. Crohn's disease (CD) affected 30%, ulcerative colitis (UC) 59%, and IBD-unclassified (IBDU) 11%.
• Infantile-IBD patients showed higher rates of IBDU, poorer initial treatment response, and lower hemoglobin/albumin levels. Long-term colectomy rates were 11%, with no significant age-group differences.
• Genetic testing in 40% of patients revealed monogenic disease in 16%, associated with lower treatment response, increased surgery, and higher infection rates.

Conclusions:

• VEOIBD, including infantile-IBD, is associated with a low incidence of long-term complications and surgical interventions.
• Patients with monogenic IBD are predisposed to a more severe disease course and poorer treatment outcomes.