Food Protein-Induced Enterocolitis Syndrome: a Comprehensive Review

  • Amanda Agyemang
  • Anna Nowak-WegrzynEmail author


Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy that has been well-characterized clinically, yet it is still poorly understood. Acute FPIES is characterized by vomiting 1–4 h and/or diarrhea within 24 h after ingestion of a culprit food. Chronic FPIES is the result of chronic exposure to an offending food that can result in chronic watery diarrhea, intermittent vomiting, and failure to thrive. FPIES typically presents in infancy and self-resolves by school age in most patients. Adult-onset FPIES is rare, but it has been reported. Cow’s milk and soy are the most common triggering foods in infants in the US, and as solids are introduced in the diet, FPIES reactions to grains (rice, oat) increase in prevalence. Variability in common trigger foods exists depending on the geographical origin—for example, fish is a frequent trigger in Spanish and Italian patients. Heavy reliance on a detailed history is required for the diagnosis as physical exam findings, laboratory tests, and/or imaging studies are suggestive and not specific for FPIES. Oral food challenges remain the gold standard for confirming diagnosis, and the challenge protocol may be for an individual depending on risk of reaction, prior reaction severity, and positive-specific IgE status. The recent development of diagnostic criteria in 2017 will serve to increase recognition of the disorder and allow for early implementation of management strategies. Acute management during reactions includes IV hydration, anti-emetics, and IV corticosteroids. Reaction prevention strategies include strict food avoidance until the physician deems a food reintroduction challenge clinically appropriate. Future efforts in FPIES research should be aimed at elucidating the underlying disease mechanisms and possible treatment targets.


FPIES Food protein-induced enterocolitis syndrome Food allergy Cow’s milk Soy Oral food challenge 



cow’s milk


food protein-induced enterocolitis syndrome


specific IgE


oral food challenge



No funding was received for this work.

Compliance with Ethical Standards

Conflicts of Interest

ANW and AA report that they have no conflict of interest.

Ethical Approval and Informed Consent

This is a review article, and ethical approval and informed consent are not applicable.


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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Jaffe Food Allergy Institute, Division of Allergy and Immunology, Department of Pediatrics, Kravis Children’s HospitalIcahn School of MedicineNew YorkUSA

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