Q:

I have 3 adults in my clinic who experience symptoms similar to FPIES. One has severe diarrhea, abdominal cramping within 3 hours of eating poultry. He required IV fluids on two occasions. One adult who developed abdominal cramping, diarrhea, fever to 103 6-8 hours after eating an omelet (Symptoms reproducible with eating omelet/scrambled eggs but tolerant of egg containing baked goods.) and one young woman with recurrent symptoms of abdominal pain, cramping and profuse diarrhea. Colonoscopy shows neutrophils and eosinophils. What is the best way to evaluate these patients? Is skin testing valuable? Patch testing to foods? Should they have epi pens?

A:

Thank you for your recent inquiry.

I have no personal experience dealing with FPIES in adults or children, and therefore am going to ask for an opinion from Dr. Anna Nowak-Wegrzyn, who fairly recently published an excellent review of this syndrome in Current Opinion in Allergy and Clinical Immunology, 2009, 9:371-377. As soon as I hear from Dr. Nowak-Wegrzyn, I will forward her response to you.

Thank you again for your inquiry.

Sincerely,
Phil Lieberman, M.D.

Below is the response we received from Dr. Nowak-Wegrzyn. Thank you again for your inquiry, and we hope this response is helpful to you.

Sincerely,
Phil Lieberman, M.D.

Response from Dr. Anna Nowak-Wegrzyn:
There are no published reports of adults with FPIES however I have heard of them anecdotally, mostly in the context of shellfish (particularly mollusk) ingestion. However, all those adults presented with profuse emesis within 2-3 hours of food ingestion. In children, I have never seen isolated diarrhea in the absence of preceding emesis or at least significant nausea and abdominal discomfort. As far as body temperature is concerned, in children, hypothermia has been reported, not fever. The first two patients are more likely to have some food-induced condition, perhaps FPIES, although their manifestations are far from “typical”. I would perform skin test and measure serum sIgE to poultry and egg, although they would most likely be negative. As far as patch testing is concerned, in our hands it has never been helpful, however, we usually perform patch testing prior to an oral food challenge following a prolonged period of food avoidance (about 18 months), which may affect the sensitivity of the patch test. If the patch test were positive, you might recommend food avoidance. If the patch test were negative, there is no guarantee that the patient will tolerate the food. The most definitive approach would be to perform the supervised oral challenge and to monitor symptoms, as well as body temperature, WBC, platelets, and blood stool. We do not prescribe epi pens for FPIES patients, however, once we establish FPIES diagnosis, we provide a letter explaining FPIES and outlining the treatment plan for acute reactions which emphasizes rapid intravenous hydration and a single dose of methylprednisolone. I might prescribe EpiPen if I found evidence of sIgE and there was h/o severe reactions with hypotension.
 
Regarding your third patient, it is difficult to assess her based on the limited information. It sounds like she has intermitted GI symptoms not related to any specific food ingestion. The results of colonoscopy are not typical for an allergic process, eosinophils are frequently seen in the colon. I would like to rule out IBD, celiac disease, as well as irritable bowel syndrome before delving into food allergy. The approach might be modified based on the additional atopic features and family history.
 
Kind regards,
 Anna Nowak-Wegrzyn, M. D.
Associate Professor of Pediatrics
Jaffe Food Allergy Institute
Mount Sinai School of Medicine


Dr. Nowak-Wegrzyn sent the following addendum to her response. Thank you again.

Sincerely,
Phil Lieberman, M.D.

Addendum to Response from Dr. Anna Nowak-Wegrzyn:
I will follow up with the refs once I get back to the office.

I guess the only other suggestion for Dr. Rolfes-Lo is to attempt an elimination diet for 3-4 weeks as an empiric approach to food allergy. We usually eliminate the big players: milk/dairy, soy, egg, wheat, fish, and nuts and assess clinical response as well as repeat biopsy.

Best,
Anna Nowak-Wegrzyn, M.D.

AAAAI - American Academy of Allergy Asthma & Immunology