Food Protein Induced Enterocoloitis Syndrome (FPIES)

Food protein induced enterocolitis syndrome (FPIES) is an under-recognised, and frequently misdiagnosed syndrome. It has been described as a 'weird food allergy'. It is characterised by severe protracted diarrhoea and/or vomiting, and is frequently associated with sepsis-like symptoms, such as pallor and/or lethargy. Symptom onset is usually soon after ingestion of the causal food protein. 

Although typically ascribed to cow’s milk and soy, FPIES has also been described after the ingestion of a wide range of food proteins. FPIES is thought to represent a severe cell-mediated, gastrointestinal food hypersensitivity.   

The presentation of the syndrome varies from mild (e.g. non-dehydrating vomiting and/or diarrhoea) to severe symptoms. Symptoms may change rapidly and manifest as a state of dehydration. Hypovolaemic shock is present in up to 20% of cases. Understandably, a combination of vomiting, lethargy and resulting acidosis leads to a primary diagnosis of sepsis. In this clinical scenario, the dietary history may not receive prominence, with the result that the syndrome recurs with each subsequent ingestion of the food protein. Failure to recognise the link with diet may lead to multiple intensive or high-care admissions, due to supposed recurrent sepsis. The incidence and prevalence of FPIES is not known. 

Common triggers include: Cow's milk, soy and fish. Many other foods have also been linked to the syndrome.
 
For a recent summary of FPIES, please see attachment below.

FPIES youtube Video


 

FPIES - YouTube Video

  

More take home points:
  • FPIES pathogenesis is probably non-IgE-mediated, so culprit food-specific IgE investigations are often negative;

  • The period of life during which FPIES appears most frequently is the first 9 months after birth;

  • The most frequent culprit food (except in Australia) is CM;

  • The most frequent solid food that induces FPIES in Italy is fish, while worldwide it is rice;

  • Common FPIES symptoms are vomiting, hypo-reactivity, hypotonia and sometimes diarrhea;

  • The evolution of FPIES is benign in a few hours; but seldom immediately as is the case in IgE-mediated food allergy

  • Diagnostic criteria consist of the occurrence of at least two typical episodes;

  • There is often a diagnostic delay – as many as six episodes could take place before the situation is clarified;

  • Acute therapeutical measures are iv. fluids and cortisone;

  • Dietetic measures consist of eliminating the culprit food from the patient's diet;

  • As far as prognosis is concerned, there is a good chance of achieving tolerance at 18 months if the responsible food is CM; however, there aren't enough data for other foods.


Updated Aug 2011
 
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George du Toit,
29 Jul 2009, 12:06
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