Food Protein Induced Enterocolitis Syndrome (FPIES)

Author: V. Dimov, M.D., Allergist/Immunologist and Assistant Professor, University of Chicago
Reviewer: S. Randhawa, M.D., Allergist/Immunologist, Fort Lauderdale, FL

What is FPIES?

FPIES is a rare (incidence 0.34%) non-IgE mediated condition (negative skin tests and sIgE) in breast fed infants. Food Protein Induced Enterocolitis Syndrome (FPIES) is a non-IgE mediated immune reaction in the gastrointestinal system to one or more specific foods, commonly characterized by profuse vomiting and diarrhea. Poor growth may occur with continual ingestion of the offending foods. Upon removing the problem food, all FPIES symptoms generally subside.

Pathology: What causes FPIES?

The mechanism of FPIES remains undefined, although tumor necrosis factor alpha is thought to play a role. Mediators released by T lymphocytes result in inflammation and vascular leakage in this non-IgE-mediated reaction. TNFα has also been implicated in food protein-induced enterocolitis (FPIES).

Adult non-IgE reactions to shellfish may be the same process as pediatric FPIES. Milk protein enteropathy looks similar to celiac disease, but it usually self-limiting.

Deficit in TGF-beta (and its receptors) may be important in development of FPIES. Increased TNF-alpha also seems to be important for FPIES.

Clinical features: When to suspect FPIES?

FPIES presents with vomiting and diarrhea, and may progress to shock. It starts early in infancy.

In a large birth cohort from Israel, the cumulative incidence for FPIES was 0.34%.

The most common symptoms were:

- recurrent vomiting (100%)
- lethargy (77%)
- diarrhea (25%)
- pallor (14%)
- bloody diarrhea (4.5%)

All patients had FPIES within the first 6 months of life.

By the age of 3 years, 90% of the patients had recovered. There is 50% resolution per year for milk-induced FPIES.

There were no concomitant reaction to soy.

The prevalence of FPIES is significant, and its clinical presentation is distinct from that of IgE-cow's milk allergy (CMA).

Most patients with FPIES recover, although a proportion might convert to IgE-CMA. The likelihood for a cross-reactivity to soy in this population was less than previously estimated.

How to diagnose FPIES?

Unfortunately, there is no diagnostic test for FPIES such as skin testing, serum specific IgE (sIgE) or patch testing.

Food challenges in FPIES

Diagnostic tests are lacking in food protein-induced enterocolitis (FPIES), a non–immunoglobulin E, presumably T cell–mediated food allergy disorder. FPIES typically presents in infancy with profuse vomiting and diarrhea 2 to 4 hours after ingestion of the allergen, combined with a left-shift in peripheral blood leukocytes, occasionally causing profound dehydration, hypotension, and lethargy. Chronic exposure results in failure to thrive and hypoalbuminemia. FPIES is elicited commonly by milk and soy protein, oat, rice, and other foods. Oral food challenge (OFC) is performed to follow tolerance development in FPIES, but protracted emesis and dehydration necessitate fluid resuscitation in up to 50% of reactive challenges. Therefore, OFC to confirm the diagnosis of FPIES can be associated with high risk (Annals of Allergy, Asthma & Immunology, 109:3, 221-222, Sep 2012, http://buff.ly/P4zn6d).

References

FPIES: Food Protein Induced Enterocolitis Syndrome. Patient Information: http://bit.ly/qad8q


The prevalence and natural course of food protein-induced enterocolitis syndrome to cow's milk:
A large-scale, prospective population-based study. J Allergy Clin Immunol. 2011 Mar;127(3):647-653.e3.

Related reading

Understanding FPIES Through Moms Sharing Their Stories. Nutricia Neocate blog, 2011.
International Association for Food Protein Enterocolitis (IAFFPE)
Food protein-induced enterocolitis syndrome (FPIES) - AAAAI Ask the Expert addresses clinical questions, 2012.
Possible food protein-induced enterocolitis syndrome (FPIES) - AAAAI Ask the Expert, 2013. http://bit.ly/16lcfY8

Published: 06/28/2010
Updated: 03/16/2013