Reviews and feature article
International consensus guidelines for the diagnosis and management of food protein–induced enterocolitis syndrome: Executive summary—Workgroup Report of the Adverse Reactions to Foods Committee, American Academy of Allergy, Asthma & Immunology

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Food protein–induced enterocolitis (FPIES) is a non-IgE cell- mediated food allergy that can be severe and lead to shock. Despite the potential seriousness of reactions, awareness of FPIES is low; high-quality studies providing insight into the pathophysiology, diagnosis, and management are lacking; and clinical outcomes are poorly established. This consensus document is the result of work done by an international workgroup convened through the Adverse Reactions to Foods Committee of the American Academy of Allergy, Asthma & Immunology and the International FPIES Association advocacy group. These are the first international evidence-based guidelines to improve the diagnosis and management of patients with FPIES. Research on prevalence, pathophysiology, diagnostic markers, and future treatments is necessary to improve the care of patients with FPIES. These guidelines will be updated periodically as more evidence becomes available.

Key words

Food protein–induced enterocolitis syndrome
guidelines
non–IgE-mediated food allergy

Abbreviations used

AAF
Amino acid–based formula
CBC
Complete blood cell count
CM
Cow's milk
FPIES
Food protein–induced enterocolitis syndrome
FTT
Failure to thrive
OFC
Oral food challenge
sIgE
Specific IgE
SPT
Skin prick test
TD
Threshold dose

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This project has been sponsored by The International FPIES Association.

Disclosure of potential conflict of interest: A. Nowak-Węgrzyn has a board membership with Merck; has consultant arrangements with Aimmune and Nestle; is employed by the Icahn School of Medicine; has received grants from DBV, Food Allergy Research and Education (FARE), the National Institutes of Health (NIH), and Thermo Fisher; has received payment for lectures from Nestle; has received payment for manuscript preparation from Nestle; has received royalties from UpToDate; has received payment for development of educational presentations from Annenberg Center; and is the chair of the Medical Advisory Board for the International FPIES Organization. M. Chehade is a member of the Medical Advisory Board for the International FPIES Association; is a member of the Medical Advisory Panel for the American Partnership for Eosinophilic Disorders; has consultant arrangements with Actelion, Receptos, and Shire; and has received grants from Nutricia and Regeneron. M. Groetch has received payment for lectures from Nutricia North American and has received royalties from UpToDate. J. M. Spergel is on the Scientific Advisory Boards for DBV Technology and Danone Nutricia; has consultant arrangements with GLG; has received grants from the NIH, Aimmune Therapeutics, and DBV Technology; has received payment for lectures from MEI; has received royalties from UpToDate; and has received stock/stock options from DBV Technology. R. A. Wood is employed by Johns Hopkins University; has received grants from the NIH, Astellas, DBV, and Aimmune; and has received royalties from UpToDate. K. Allen has consultant arrangements with Nestle, Thermo Fisher, Aspencare, and Nutricia. D. Atkins is on the Medical Advisory Board for the American Partnership for Eosinophilic Disorders, is on the Data Monitoring Safety Board for MILES study for DBV Technologies, has received payment for lectures from Regeneron, and has received travel support from Monsanto. A. Barad has received payment for lectures from Abbott Nutrition. C. Berin has received grants from the NIH. A. W. Burks has received grants from the Food Allergy & Anaphylaxis Network, the NIH, and the Wallace Research Foundation; has received personal fees from FARE, the NIH AITC Review Panel, the NIH HAI Study Section, World Allergy Organization, Aimmune Therapeutics, Epiva Biosciences, Genentech, Merck, Stallergenes, Valeant Pharmaceuticals North America, PPD Development, Allertein, and Sanofi US Services; and has received nonfinancial support from Regeneron Pharmaceuticals. J.-C. Caubet is employed by Geneva University Hospitals and has received payment for lectures from Thermo Fisher. A. Fiocchi has received a grant from the International FPIES Association, has a board membership with Ferrero, and is employed by Ospedale Pediatrico Bambino Gesù. K. Grimshaw has consultant arrangements with Reacta Biotech, has received payment for lectures from Danone and Abbott, and has received travel support from Abbott. R. Gupta has consultant arrangements with Kaleo and BEFORE Brands and has received grants from Mylan Specialty, Aimmune, and United Healthcare Group. S. A. Leonard is on the Medical Advisory Board for FARE, has received a grant from the FARE Clinical Network, has received payment for lectures from Allergy and Asthma Medical Group and Research Center, and has received travel support from the American Academy of Allergy, Asthma & Immunology. J. Lightdale has consultant arrangements with Perrigo Nutritionals, Norgine, and Medtronic; has received grants from Abbvie; has received payment for lectures from Mead-Johnson; has received royalties from 5-Min Pediatric Consult; and has received payment for development of educational presentations from Mead-Johnson and Perrigo Nutritionals. S. Mehr has received grants from the Allergy and Immunology Foundation of Australasia and has received payment for lectures from the Immune Deficiency Lecture Series. A. Muraro has consultant arrangements with MEDA, Novartis, and Menarini; is employed by Padua University Hospital; and has received payment for lectures from MEDA and Menarini. S. Noone has consultant arrangements with Aimmune, is employed by Icahn School of Medicine at Mount Sinai, and has received royalties from Up To Date. H. A. Sampson has received royalties from UpToDate and was employed 60% of his time as a Professor of Pediatrics and Director of the Jaffe Food Allergy Institute by the Icahn School of Medicine at Mount Sinai and 40% of his time as the Chief Scientific Officer at DBV Technologies. S. H. Sicherer has received grants from the National Institute of Allergy and Infectious Diseases and Food Allergy Research and Education and has received royalties from UpToDate. P. J. Turner has consultant arrangements with Reacta Biotech and the UK Food Standards Agency; is employed by Public Health England and Imperial College London; has received grants from the Medical Research Council, NIHR/Imperial BRC, EU FP7 Programme, and UK Department of Health; and has received travel support from the National Institute for Health and Care Excellence. C. Venter has consultant arrangements with Danone and Mead Johnson and has received payment for lectures from Nestle. A. Westcott-Chavez is a board member for the International FPIES Association. M. Greenhawt has received a grant from the Agency for Healthcare Research and Quality (1K08HS024599-01, Career Development Award); has received travel support from the National Institute of Allergy and Infectious Diseases and the Joint Taskforce on Allergy Practice Parameters; is on the scientific advisory council for the National Peanut Board; has consultant arrangements with the Canadian Transportation Agency, Nutricia, Nestle, Aimmune, Kaleo Pharmaceutical, Monsanto, and Intrommune Pharmaceutical; is an Associate Editor for the Annals of Allergy, Asthma, and Immunology for the American College of Allergy, Asthma & Immunology; and has received payment for lectures from Reach MD, Thermo Fisher Scientific, New York Allergy and Asthma Society, UCLA/Harbor Heiner Lectureship, Medscape, the Aspen Allergy Society, the European Academy of Allergy and Clinical Immunology, the Canadian Society of Allergy and Clinical Immunology, the Pennsylvania Society for Allergy and Immunology, and American College of Allergy, Asthma & Immunology. The rest of the authors declare that they have no relevant conflicts of interest.