Food Protein-Induced Enterocolitis Syndrome (FPIES)

Food Protein-Induced Enterocolitis Syndrome (FPIES), is a delayed, non-IgE-mediated food allergy typically presenting in infancy or early childhood. In recent years, adult-onset FPIES has also been increasingly recognized. Symptoms of FPIES usually occur one to four hours after ingestion of the food allergen and include repetitive vomiting, along with lethargy, color change and/or diarrhea. Severe cases can progress to dehydration and shock. The main diagnostic criteria for FPIES, is repetitive, projectile vomiting, however in adult-onset FPIES, vomiting is absent in up to 40% of adults, while severe abdominal pain is the most common finding, associated with nausea or diarrhea occurring one to six hours after ingestion of the food allergen.

In FPIES, any food can be a trigger, but the common food triggers can vary based on age and geographic location. In pediatric FPIES, common triggers include cow’s milk and grains (oat, rice), and more recently, egg and peanut. Seafood is a common trigger in adult-onset FPIES. Most individuals with FPIES have one to two food triggers, but it is possible to have FPIES reactions to multiple foods.

Diagnosing FPIES
Symptoms of FPIES can overlap with other medical conditions, so a diagnosis is not always obvious. Because there are no laboratory or skin tests to confirm a delayed non-IgE-mediated food allergy, diagnosing FPIES is based on history, symptoms and physical examination. An allergist / immunologist will take a detailed history, including foods eaten and a timeline of reactions. In recent years, a subtype of FPIES known as “atypical” FPIES has been recognized where patients have evidence of IgE to their food trigger on skin or blood testing but present with delayed symptoms associated with FPIES. A small percentage of these patients may transition from having FPIES to developing immediate IgE allergy symptoms to their FPIES food trigger.

Treatment & Management
Management of FPIES is to strictly avoid the food(s) that trigger an allergic reaction. This requires careful attention to the diet of a patient with FPIES. If an FPIES reaction does occur, treatment includes the administration of oral or intravenous fluids to counteract fluid loss from vomiting and diarrhea. Additionally, ondansetron can be administered to stop the vomiting. For a severe FPIES reactions, steroids may also be given, in addition to fluids and ondansetron.

Prior to prescribing or administering ondansetron, patients should be asked if they have any known risk factors for a condition called QTc prolongation, such as a history of cardiac arrhythmia or renal disease leading to electrolyte abnormalities (low potassium or low magnesium). Medications that may increase risk of QTc prolongation include; antiarrhythmics (e.g. amiodarone, procainamide, quinidine, sotalol), antibiotics (e.g. macrolides, fluoroquinolones), antidepressants (e.g. SSRIs (citalopram, escitalopram) or tricyclic antidepressants (amitriptyline)), antiemetics (e.g. ondansetron, domperidone), antifungals (e.g azoles (fluconazole), pentamidine)), methadone, hydroxychloroquine or donepezil.

If you or your child have FPIES, you will need to be closely followed by a physician to discuss what foods are safe and when it may be time to determine if FPIES has resolved. An oral food challenge can be performed to assess for resolution of FPIES usually within six to 18 months from the last FPIES reaction, depending on the food and past reaction severity. With proper medical attention and a personalized dietary plan, patients with FPIES can continue to thrive and avoid potential growth and/or nutritional deficiencies.

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

For more information on FPIES please contact The International FPIES Association (I-FPIES).

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4/3/2024