Food Protein-Induced Enterocolitis Syndrome (FPIES)

Food Protein-Induced Enterocolitis Syndrome (FPIES) Posted By:

Food protein-induced enterocolitis syndrome (FPIES) is a non-immunoglobulin (Ig) E-mediated food allergy that typically presents in infants. The most common foods known to trigger FPIES include (in order of prevalence): rice, oats, cow's milk, soy, eggs, fish, fruits, and vegetables. Approximately 60% of infants have a single trigger food, but 33% may react to two or three foods, and 10% react to more than three foods.

The overall prevalence of FPIES in US infants is about 0.51%. Due to the delayed development of symptoms, proper recognition and diagnosis of FPIES can be challenging. Additionally, there are many different types of FPIES, including acute, chronic, adult-onset, atypical, and FPIES related to exclusively breastfed infants. This article will review the clinical presentation, diagnosis, and management of acute FPIES.

Acute FPIES is characterized by repetitive projectile vomiting within 1 to 4 hours of ingestion of a causative food; diarrhea usually develops within 24 hours. Other common symptoms or complications include lethargy, pallor, hypotonia, dehydration, hypothermia, and hypovolemia; shock is also possible. Acute FPIES usually presents in otherwise healthy and growing older infants, but can present in older children as well. Infantile FPIES typically resolves by school age.

Diagnosis of FPIES is clinical, based on recognition of its classic historical findings and symptoms. There is no diagnostic test or biomarker related to the condition. In contrast with classic IgE-mediated food allergy, serum-specific IgE testing and skin-prick testing to foods will be negative, as the disorder is non-IgE-mediated and usually involves delayed T-cell responses.

First-line management of FPIES is avoidance of offending foods. Proper management of reactions includes fluid rehydration and administration of ondansetron as an anti-emetic. Due to the risk of hypovolemia and shock, many infants with FPIES reactions are managed in the hospital, where fluids can be administered intravenously. Adrenaline or epinephrine have no place in the management of FPIES.

Because of the lack of awareness of FPIES in the community, many patients and parents are provided with "FPIES letters" to provide to emergency department staff should they need to seek medical care. These letters serve to provide the attending physician or provider with information about acute FPIES and management.

Since many children outgrow FPIES by school age, oral food challenges are typically done in the specialty office (allergist) or hospital. The consensus is to consider attempting oral food challenge when there has been at least 12 months since the child's most recent reaction. During oral food challenges, patients are given appropriate small or split doses and observed for reaction for 4 to 6 hours afterwards.

The biggest unmet needs in FPIES involve lack of awareness of the condition amongst medical providers, lack of diagnostic biomarkers, and lack of treatment options to quicken resolution of the condition. For now, what we can do is offer reassurance to parents that resolution of the condition usually occurs by school-age, and that most children have an excellent prognosis without any long-term effects.

  • Nowak-Wegrzyn A, Berin MC, Mehr S. Food protein-induced enterocolitis syndrome. J Allergy Clin Immunol Pract. 2020;8:24-35.


Filed under: Allergy/Immunology

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