Food Protein-Induced Enterocolitis Syndrome

From Practice to Theory

Stefano Miceli Sopo; Monica Greco; Serena Monaco; Salvatore Tripodi; Mauro Calvani


Expert Rev Clin Immunol. 2013;9(8):707-715. 

In This Article

FPIES Definition and Diagnostic Criteria

FPIES is an important cell-mediated food hypersensitivity, probably non-IgE-mediated and most commonly caused by CM and soy.[1] There could be other culprit solid foods, such as fish, rice, egg, legumes, poultry, but they are less frequent. Acute FPIES (the most frequent form) is characterized by repetitive and severe vomiting, pallor, hyporeactivity and is less frequently accompanied by diarrhea that can lead toward dehydration and shock in a 10−20% of patients. FPIES clinical findings are listed in Table 1. FPIES can also manifestate itself in a chronic form. This was most frequent in the past years, when it was used to be called 'CM protein intolerance' and it was often diagnosed by gastroenterologists. Nowadays the chronic form is very rare; our clinical records of 55 children report that only three patients are affected by the chronic form.

In FPIES some typical laboratory findings such as neutrophilia and thrombocytosis can be found, especially in the acute form, but their documented presence is not indispensable for the diagnosis. This last one is based on five clinical criteria, which must be fully satisfied to make the diagnosis. They were already listed by Powell in 1986[2] and recently Leonard et al.[1] reviewed them and submitted a new version. These criteria are very important and it is necessary to understand their relevance. Now we would like to briefly make some comments concerning the original criteria of Powell[2] and the changes proposed by Leonard et al.;[1] Table 2 summarizes the above criteria and our proposal.