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Update on Food Protein–Induced Enterocolitis Syndrome (FPIES)

  • Pediatric Allergy and Immunology (W Dolen, Section Editor)
  • Published:
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Abstract

Purpose of Review

Food protein–induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy characterized by delayed, and potentially severe, gastrointestinal symptoms. Since the advent of a specific diagnostic code and establishment of diagnostic guidelines, our understanding of this condition has grown.

Recent Findings

FPIES affects patients from early infancy into adulthood. Any food can be a trigger, and common culprit foods vary geographically and by age. An understanding of the complex underlying immune mechanisms remains elusive, although studies show pan-leukocyte activation, cytokine release, and increased gastrointestinal permeability. Management involves trigger avoidance, and patients may benefit from the support of a dietitian to ensure adequate nutrient intake. Tolerance develops over time for most children, but due to the risk of severe symptoms, re-introduction of a suspected FPIES trigger is recommended only under supervision at an oral food challenge. Studies continue to evaluate the optimal challenge protocol. Caregivers of children with FPIES report high levels of anxiety and stress, which is attributed to the dramatic symptomatology, dietary restrictions, nutritional concerns, lack of confirmatory diagnostic tests, and limited tools for management of reactions.

Summary

Our understanding of the FPIES diagnosis has improved over the last few decades, but there remain opportunities, particularly regarding discerning the pathophysiology and best management practices.

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Abbreviations

FPIES:

Food protein-induced enterocolitis syndrome

IgE:

Immunoglobulin E

IL-2:

Interleukin-2

IL-8:

Interleukin-8

IL-17:

Interleukin-17

OFC:

Oral food challenge

RNA:

Ribonucleic acid

TGF-β:

Transforming growth factor-β

TH2:

T helper cell type 2

TH17:

T helper cell type 17

TNF-α:

Tumor necrosis factor-α

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Acknowledgements

Dr. Baker was supported in part by the Louis and Rachel Rudin Foundation.

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Correspondence to Mary Grace Baker.

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Baker: None. Berin: Reports grants to her institution from the National Institute of Allergy and Infectious Diseases. Sicherer: Reports royalty payments from UpToDate and from Johns Hopkins University Press; grants to his institution from the National Institute of Allergy and Infectious Diseases, from Food Allergy Research and Education, and Pfizer; and personal fees from the American Academy of Allergy, Asthma and Immunology as Deputy Editor of the Journal of Allergy and Clinical Immunology: In Practice, outside of the submitted work.

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Baker, M.G., Cecilia Berin, M. & Sicherer, S. Update on Food Protein–Induced Enterocolitis Syndrome (FPIES). Curr Allergy Asthma Rep 22, 113–122 (2022). https://doi.org/10.1007/s11882-022-01037-y

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