The main topic at this month’s SEED (SouthEast Eosinophilic Disease) Center Journal club was Food Protein-Induced Enterocolitis Syndrome (FPIES) with two featured articles:
- J Allergy Clin Immunol In Practice 2013; 1: 317-22. Review
- J Allergy Clin Immunol In Practice 2013; 1: 343-9. Original Study
The review covers the key issues including presentation, diagnosis, differential diagnosis, outcome and management. Some of the key points:
- FPIES is characterized by repetitive emesis (often to the point of dehydration and lethargy) and sometimes diarrhea. It typically starts within the first 3-6 months of life.
- Trigger foods are most commonly milk, then soy, then grains (rice cereal). FPIES in exclusively breastfed babies is extremely rare.
- FPIES is a non-IgE mediated reaction; thus testing with skin prick tests or serum for food-specific IgE has poor utility.
- Differential diagnosis (Table E4): gastroenteritis/food poisoning, sepsis, anaphylaxis, inborn errors of metabolism, intussception, Hirschsprung’s, necrotizing enterocolitis, and proctocolitis
- Variable time to resolution (Table E6): Cow’s milk resolution up to 60% resolution by 10 months, though some studies report 60% resolution at 3 years. Soy resolution as much as 90% by 10 months of age (less in other studies). Solids -resolution in 67% by 3 years.
- Management: Avoid trigger foods. If supplementing breastmilk, consider hydrolyzed (or amino acid based) formula. Conduct food challenges in supervised medical setting (often inpatient). Acute management: Consider intravenous fluids and methylprednisolone (1 gm/kg) during bouts
2nd Article: Retrospective chart review of 462 patients with FPIES from CHOP (Philadelphia). Inclusion criteria: “classic reaction of prolonged vomiting and diarrhea that occurred 2-6 hours after ingestion of the food.”
- Diarrhea occurred in about 50%.
- Mean age of onset was 7 months for milk or soy compared with 12 months for grains
- 43% of patients with milk-triggered FPIES react to soy as well
- 42% of patients with a grain trigger react to two or more grains
- More than 85% outgrew FPIES by 5 years of age. 35% outgrew their FPIES by age 2, 70% by age 3, and 80% by age 4.
Journal club discussion:
- It was noted in the group discussion that FPIES in adults is most often triggered by shellfish/fish and eggs.
- FPIES does not “run” in families. Though, atopic patients have increased risk. (As an aside: If you have diarrhea, it might be genetic –it might run in your jeans.)
- The nomenclature of FPIES is problematic. How come only ~50% have diarrhea if this is an “Enterocolitis” disorder?
- Typically, trigger foods would not be reintroduced for a minimum of 12-18 months after last exposure/reaction.
Take home message: FPIES is a clinical diagnosis. Be careful with oral challenges.
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