Food protein-induced enterocolitis syndrome in children: An updated review on pathogenesis, diagnosis, and management

Food protein-induced enterocolitis syndrome (FPIES) is a severe non-IgE-mediated food allergy with unclear prevalence and pathophysiology. FPIES typically presents in the first year of life but can present later in adulthood in an acute or chronic manner. Acute FPIES manifests 1-4 hours after ingestion of foods such as rice, oats, and cow's milk, causing repetitive vomiting, diarrhea, lethargy, dehydration, and hypovolemic shock. Interestingly, affected infants are generally mistakenly diagnosed with sepsis or viral gastroenteritis. Chronic FPIES presents with episodic vomiting, watery diarrhea, and poor growth, potentially leading to dehydration and hypovolemic shock over days to weeks in infants under 3 months who are receiving cow's milk or soy formula. Unlike classic food allergies, FPIES does not present with rash, urticaria, or respiratory symptoms, and specific blood IgE and skin prick tests are negative for the culprit food. While most cases resolve by the age of 3-5 years, some may persist into adulthood, particularly in individuals allergic to cow's milk and soy. Aside from avoiding offending foods, no specific treatment exists. The first international consensus guideline for diagnosing and managing FPIES was established in 2017, marking a vital advancement in the field. However, further research is needed to better understand the pathogenesis, diagnosis, and treatment options for the disease.