Multicenter food protein-induced enterocolitis syndrome (FPIES) data collection: Leveraging a REDCap FPIES registry for improved clinical outcomes.

Background: Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy typically presenting in infancy but has also been recognized in adults. FPIES is an allergic emergency due to severe vomiting occurring 1 to 4 hours after ingesting the causative food protein. Since the 2017 FPIES guidelines, no prospective data exist on the prevalence, incidence, and clinical characteristics of FPIES.

Objective: We established a multicenter FPIES registry to systematically collect clinical data and biospecimens on FPIES patients.

Methods: The FPIES registry is a US multicenter REDCap database collecting epidemiologic data to support the evolving FPIES landscape in relation to age at diagnosis, triggers and coreactivity, disease resolution, and risk of disease conversion to IgE allergy. Questionnaire and biosampling strategies have been developed using a systems biology approach to identify determinants of FPIES.

Results: The registry includes patients with physician diagnosis of FPIES (ICD-10 code K52.21) from January 2015. Longitudinal REDCap instruments for FPIES data collection include: age at first reaction, age at diagnosis, reaction timing, symptoms, treatment, medical care or hospitalization for reaction, dietary triggers, atopic comorbidities, family history of atopy and FPIES, oral food challenge procedures (eg, intravenous line placement, dosing protocol, observation period, reaction timing, symptoms and treatment), age at food trigger resolution, food-trigger IgE, cases converting from atypical FPIES to IgE-mediated food allergy, and sample collection data.

Conclusions: The registry will provide a multicenter repository of data and biospecimens, enabling identification of clinical determinants and phenotypes of FPIES, better understanding of conversion risks, and identification of biomarkers and mechanisms associated with FPIES.