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

Abstract

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.

Keywords: Food allergy; Allergy; Children; Enterocolitis; Protein-induced enterocolitis

Core Tip: Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated hypersensitivity that affects the gastrointestinal tract following ingestion of trigger foods. It typically presents in infancy and is characterized by repetitive, protracted vomiting that begins approximately 1–4 h after ingestion of specific foods. The pathophysiology involves predominantly cell-mediated immune mechanisms. Patients are often misdiagnosed as having sepsis, viral gastroenteritis, or a surgical abdomen. Management includes both acute care during the episodes and long-term dietary modifications and nutritional support. Most children recover from the disease. Multicenter randomized controlled trials are required to build evidence-based guidelines for the diagnosis, acute treatment, and oral immunotherapy. Additionally, awareness of FPIES remains limited among pediatricians and emergency care providers; therefore, more studies focusing on this condition are warranted.