Management of Food Protein-Induced Enterocolitis Syndrome (FPIES): Current Approach and Future Needs

Abstract

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE, cell-mediated food allergic gastrointestinal disorder triggered by ingestion of food proteins (cow milk, soy, rice, oat, egg, and others). The acute FPIES begins approximately 1–4 h after food ingestion with profuse, repetitive emesis that is accompanied by lethargy and pallor and may be followed later by diarrhea; 15% will require hospitalization because of hypotension and hemodynamic instability due to severe dehydration. A chronic FPIES is less frequent and develops from few days to 4 weeks after the offending food has been introduced and fed daily. Chronic FPIES presents with intermittent emesis, bloody diarrhea, dehydration, weight loss, and failure to thrive and has been only reported in young infants fed with cow’s milk and soy-based formulas. Acute FPIES to seafood has been reported in older children and in adults. The majority of patients react to a single food; however, FPIES can be triggered by more than one food, especially between cow milk/soy and rice/oats. Diagnosis of FPIES is based on the recognition of clinical manifestations, exclusion of alternative etiologies, and a physician-supervised oral food challenge (OFC). OFC is the gold standard; however, most patients do not need to undergo confirmatory OFC, especially if they have a history of severe reactions and become asymptomatic following elimination of the suspected food from diet until tolerance through OCF is proven. Management of FPIES is based on elimination of the trigger food in the diet, medical treatment of acute reactions due accidental exposure (intravenous hydration, methylprednisolone, ondansetron), nutritional counseling regarding avoidance of trigger foods, and introduction of the complementary foods and periodic oral food challenges to evaluate for resolution. The first international consensus guidelines on diagnosis and management of FPIES published in 2017 provide practical support for the providers caring for patients with FPIES.