Evolving a Single Institution’s Approach to Very Early Onset Inflammatory Bowel Disease (VEO-IBD) through Literature Review and Collaborative Efforts

Abstract

Objective: Very Early Onset Inflammatory Bowel Disease (VEO-IBD) is defined as a diagnosis of IBD before 6 years of age. VEO-IBD often follows a more recalcitrant disease course and can be associated with underlying Inborn Errors of Immunity (IEI). Methods: We describe our single institution experience in caring for patients with VEO-IBD as a tertiary referral center for pediatric IBD, how we utilized this most recent literature review to provide self-assessment in workup, and how we transformed our approach to VEO-IBD care through collaboration, using a stepwise laboratory and clinical paradigm for this vulnerable cohort of IBD patients. Results: A retrospective review of 34 patients was performed with description of clinical phenotypes, tissue pathology, clinical management and outcomes. The majority of our VEO-IBD patients presented with colonic Crohn's disease. A high percentage of patients were refractory to anti-Tumor Necrosis Factor (TNF) therapy. Only 26/34 (76%) of our VEO-IBD patients had any immunological testing done and genetic testing was performed on only 5/34 patients (15%). Discussions: Our review process identified a need for expedited standardized immune workup for this population at our institution. Based upon this introspective work, an algorithm for immunological and genetic evaluation at time of diagnosis was developed, aimed at creating a multi-disciplinary approach for the comprehensive evaluation of this at-risk patient population.