Food Protein-Induced Enterocolitis Syndrome

Area of Science:

• Pediatric Allergy and Immunology
• Gastroenterology
• Immunology

Background:

• Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy.
• It typically presents in infancy with projectile vomiting, diarrhea, and systemic symptoms like lethargy and hypotension.
• Increasing recognition of FPIES onset in older individuals.

Purpose of the Study:

• To review current concepts in the epidemiology, pathophysiology, diagnosis, and management of FPIES.
• To highlight diagnostic challenges and current management strategies.
• To discuss the obscure pathophysiology and lack of tolerance-inducing strategies.

Main Methods:

• Review of current literature on FPIES.
• Analysis of epidemiological data, including incidence and prevalence.
• Synthesis of information on diagnostic criteria and management approaches.

Main Results:

• FPIES affects approximately 0.51% of US infants, with a cumulative incidence ranging from 0.015% to 0.7%.
• Diagnosis is challenging due to delayed symptom onset (1-4 hours post-ingestion), absence of typical allergic signs, and often unrecognized food triggers.
• Pathophysiology remains unclear, though innate immune activation is observed.

Conclusions:

• FPIES diagnosis relies on clinical recognition due to the lack of specific biomarkers.
• Management involves strict food trigger avoidance, prompt treatment of accidental exposures, and supervised oral food challenges for monitoring resolution.
• Further research is needed to elucidate FPIES pathophysiology and develop strategies to accelerate tolerance development.