Introduction
Food protein-induced enterocolitis syndrome (FPIES) is a non IgE-mediated food allergy. Its incidence is estimated between 0.015-0.7%,1–4 corresponding to 19-31% of food allergies diagnosed in infants and children.3,5,6 In the absence of biomarkers, the diagnosis of FPIES is based on clinical presentation. International diagnosis criteria have recently been proposed to improve its diagnosis.7 Acute FPIES is defined by typical repetitive vomiting starting 1 to 4 hours after ingestion of the culprit food, in association with at least 3 minor criteria.7 These include some other episode of repetitive vomiting after eating the same or different foods, lethargy, pallor, the need for an emergency department visit or intravenous fluid support; diarrhea, hypotension or hypothermia.7Chronic FPIES occurs when the food is regularly consumed, and is mainly reported with cow’s milk (CM) and soy formula ingestion. The diagnosis of chronic FPIES is based on the presence of intermittent emesis, chronic diarrhea, poor weight gain or failure to thrive, which improve after several days to weeks of exclusion of the offending food. After a period of avoidance, acute typical symptoms occur upon reexposure.8 Severe forms of acute FPIES may lead to dehydration, and hypovolemic shock is reported in 15% to 33% of acute FPIES cases.9–11 IgE sensitization to the culprit food is unusual but may be observed in atypical FPIES.7 Although oral food challenges (OFC) are not necessary for diagnosis when the typical symptoms are present, they are useful in doubtful cases to confirm the diagnosis.7The offending foods depend on geographic origins.8 The most frequent culprit foods are CM in Europe and North America,6,12–18 soya, rice and grains in North America and Australia,2,4,10,17,19 and fish in Mediterranean countries.13,20 Resolution of FPIES is expected by school age in the majority of cases.8 OFCs are performed to assess tolerance to the food in question, generally 12-18 months after the last reaction.7
In this study we aimed to describe FPIES in a large population of French children, using international diagnosis criteria, and to describe its natural history.7