SEED Journal Club: FPIES

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.”

Key findings:

  • 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.

Related blog posts:

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