Food protein-induced enterocolitis (FPIES)

Introduction

Food protein–induced enterocolitis syndrome (FPIES) is an immunologic reaction that occurs in the digestion system. It is often unrecognized, and undiagnosed. The reaction is often delayed and may occur 1-5 hours after food ingestion. The major symptom is profuse vomiting 1 to 5 hours after food ingestion. The most common implicated foods are milk, soy, rice, and oats.

What are the symptoms of FPIES

• Vomiting (often repetitive) 1-4 hours after ingestion
• Lethargy
• Pallor

Natural history

FPIES often presents prior to 6 months of age in formulas–fed and infants with repetitive vomiting, lethargy, and dehydration 1-5 hours after food ingestion. The most common foods include milk, soy, rice, and oats. Other foods that have been reported include barley, chicken, turkey, egg, pea, peanut, sweet potato, white potato, free protein, fish, and shellfish. In 60-90% of children, FPIES dissolves by 3 years of age. However, FPIES can occur in adults, especially to shellfish. The reactions can occasionally be extremely severe with significant dehydration requiring emergency room visits, and sometimes hospitalization. FPIES can sometimes be confused with a severe infection called sepsis.

Management of FPIES

FPIES can be treated with intravenous hydration. There is no treatment that cures FPIES at this time. The mainstay of treatment is strict avoidance of the offending food. The family may need education and support regarding what foods to add to the diet since FPIES may occur to more than 1 food.

Further support/information

FPIES is a complex disorder that may seem overwhelming at times. Individuals and families may benefit from participating in support groups, as well as lay organizations such as the International FPIES Association and The FPIES Foundation. The International Consensus Guidelines for the Diagnosis and Management of FPIES was recently published (2017) in the Journal of Allergy and clinical Immunology. Click here for an FPIES fact sheet and here for an FPIES ER letter.  Additional resources include an FPIES weaning guide, FPIES co-allergies, and FPIES emergency room orders.