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.

Acute Management of FPIES

There are not many measures to take except giving iv. fluids and steroids. At this regards, Sicherer^[17] wrote: "On the basis of the only partially understood pathophysiology of the immune response (T-cell–mediated) and observations of the clinical symptoms (lethargy, dehydration, shock), several suggestions can be made for emergency care that are based on response to treatments in studies reporting results of oral food challenges. If an ingestion is known to have occurred, the patient should be instructed to present to medical attention for observation. Intravenous fluid resuscitation may be needed. For patients with a history of severe reaction and onset of any symptoms, consideration should be given for administration of corticosteroids to quell a presumed T-cell–mediated reaction, and this treatment could be considered for any patient with more than minimal symptoms. From the words of Sicherer, it can be concluded that the treatment of acute episodes of FPIES is only a practice. In fact, there are no randomized studies demonstrating the usefulness of corticosteroids and, indeed, even of iv. fluids. On this topic, Powell^[2] suggested that it could be safest to place a venous access before starting an OFC, while more recently different measures were adopted. For example, Katz et al.^[4] do not place any preventive venous line and they are used to treat patients who failed OFCs with oral rehydratation. From 2008, we do not take any prophylactic measure either, as failed OFCs resolve spontaneously. We are of course always ready to take the necessary therapeutic measures, such as we did for Bridget. This less aggressive management does not produce any worst outcomes, compared to those of other Italian allergic centers^[5] that conformed to all procedures stated by Powell.^[2] On the other hand, the majority of acute FPIES episodes usually occur at home and these patients do not receive any medical assistance at all, just as Ernst experienced three times. According to the literature, and until now, FPIES did not cause any deaths. There still are many doubts about the utility of im. epinephrine, which is strongly recommended in case of anaphylaxis; we administered it only in two serious situations, one of which was Bridget's reaction. We felt that this was a useful measure, but they are only two cases. In this regard Sicherer writes:^[17] "The reaction can include symptoms of shock that may presumably respond to epinephrine, but our experience has been that iv. hydration and steroids are the only medications typically required." We think that the role of epinephrine in the treatment of acute FPIES is not clearly known: at the current state of knowledge, it is not possible to exclude it. However, we wish to emphasize that the use of epinephrine in the therapy of acute FPIES is not part of the management officially suggested.

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Table 1. Acute and chronic FPIES clinical manifestations.
Acute FPIES	Chronic FPIES
Important and repetitive vomiting (constant)	Regurgitation, small amounts of vomit
Lethargy (frequent)	Lethargy
Hyporeactivity (frequent)	Failure to thrive
Pallor (frequent)	Dehydration
Diarrhea (inconstant and tardive)	Diarrhea, sometimes bloody
Dehydration (rare)	Abdominal distention
Hypothermia (rare)	Hypotension (rare)

Table 2. Diagnostic criteria of FPIES.
Powell, 1986²	Leonard et al., 2012¹	Miceli Sopo et al., 2013
Less than 9 months of age at initial presentation (reaction)	Less than 9 months of age at initial diagnosis	Less than 2 years of age at first presentation (frequent feature but not mandatory)
Exposure to the incriminated food elicits repetitive vomiting and/or diarrhea within 4 h without any other cause for the symptoms	Repeated exposure to causative food elicits gastrointestinal symptoms without alternative cause	Exposure to the incriminated food elicits repetitive and important vomiting, pallor, hyporeactivity and lethargy within 2–4 h. Diarrhea may be present, much less frequently and later. The symptoms last a few hours, usually less than 6 h
Symptoms limited to the GI tract	Absence of symptoms that may suggest an IgE-mediated reaction	Absence of symptoms that may suggest an IgE-mediated reaction
Avoidance of the offending protein from the diet results in resolution of symptoms	Removal of causative food results in resolution of symptoms	Avoidance of the offending protein from the diet results in resolution of symptoms
A standardized food challenge or isolated re-exposure elicits the typical symptoms	Re-exposure or oral food challenge elicits typical symptoms within 4 h	Re-exposure or oral food challenge elicits typical symptoms within 2−4 h. Two typical episodes are needed to deliver the definitive diagnosis

Box 1. Clues in favor of a specific IgE role in food protein-induced enterocolitis syndrome pathogenesis.
There are some cases with positive specific IgEs against the culprit food, even if their levels are low. In one child,, during an episode of acute FPIES caused by rice, a plasmatic cytokinic trend similar to those of IgE-mediated allergic reactions was observed, with an increase of IL-4 and a decrease of IFN-γ [10]. The fact that symptoms arise 2 h after the ingestion of the culprit food is not a typical feature either of classical IgE-mediated forms (the reaction usually appears long before 2 h) or of classical non-IgE-mediated forms (which usually manifest long after 2 h). Few patients (with positive specific IgE research) present a shift in symptoms from those of FPIES to those of gastrointestinal anaphylaxis [11]. Regarding CM FPIES, there is a report about the capability of tolerating the culprit food if well-cooked (baked) [12].