Q:

1/9/2014
I have seen a 54 yo Family Practice Physician, who was in good health until Nov 2012 when she began to notice abd pain/vomiting-- hospitalized with dehydration-- dx'd with eosinophilic gastroenteritis. Peripheral esoinophilia of 43% prior to starting prednisone therapy and she has not been able to wean herself off of it, currently on 5mg/day. Bone marrow bx was neg; PGDF-Ra, PDGF-Rb and FGF-1 mutation analysis was unremarkabl. Blood testing to foods was + to eggs only. Skin testing to foods was neg. She has a h/o bloating/diarrhea with whey protein and dairy. Overtime, she has developed GI distress (RUQ abd pain and more recently, chest tightess/wheezing/cough/rhinorrhea) to an increasing number of foods. PFTs were normal and methacholine challenge was normal. Chest sxs improved with a 2 mo trial of Advair along with escalation of prednisone dose. She has also developed hypersensitivity to various odors (sxs of chest congestion/cough/wheezing/rhinorrhea). Elemental diet was tried but the flavoring caused chest tightness). Currently, if she does not eat anything, she feels well, but if any of the foods outside of the narrow list of tolerated foods is tried, she gets RUQ and back pain (is s/p cholecystecty/ neg HIDA/ neg RUQ US) along with chest tightness. Wheat leads to fairly immediate swelling of hands and knees which can persist for days.

She was evaluated in summer 2013, she underwent w/u, which included serology for parasitic infection (Babesia Microti IgG, E. Histolytica Ab, Schistomsoma IgG, Toxocara Ab, Trichinosis Ab, Hookwork Ab, Ascaris Ab were normal. 3 stool samples for parasites were normal. Stool strongyloides culture was normal.
CBC w/o eosniophilia.
Normal peripheral blood flow cytometric immophenotyping.
Serum electrophoresis showed a very mildly elevated Alpha 2 globulin.
24 hr urine histamine and beta prostaglandin levels were normal.
Plasma IL-5 level was normal.
AST/ALT were normal
ESR was normal. Vit B12 was normal
Total serum IgE was normal
Total serum tryptase was normal.
Calcinonin level was normal.
C-ANCA/ p-ANCA RF, Myeloperoxidase Ab, Proteinase 3 Ab ere normal.
CBC with diff showed neutrophilia (pt is on systemic steroids), low monotcyte and eosinophil count.
She was treated for positive H pylori breath test.
CT entergraphy was neg for small bowel wall thickening or ascites but showed small low destiy lesions in the liver (likely simple cysts or cavernous hemangiomas). RUQ US was neg for biliary dilatation.
Aeroallergen skin prick testing (while on chronic prednisone therapy) was + to Kentucky Blue/June grass, Orchard grass, Pigweed, Russian thistle, and Sagebrush.
PFT and methacholine challenge testing was normal.

At present, it seems that we have a confirmed diagnosis of eosinophilic gastroenteritis. She had 43% peripheral eosinophilia prior to starting prednisone. BM bx, genetic mutations for clonal proliferation of eosinophils, peripheral blood flow cytometric immunotyping, and SPEP have been unremarkable. A detailed w/u for secondary causes of eosinophilia has been unremarkable (parasitic/helminthic infection; rheumatologic w/u). CT enterography was neg for any small bowel abnormality. PEG-interferon was recommended, which patient was not interested in it, given the side effect profile. She did do a trial of Hydroxyurea, again upon recommendation, but did not notice any benefits except associated headaches, so this was discontinued. She has mild rhinitis sx and aeroallergen skin testing positive to a few grasses and weeds (test performed while she was on chronic prednisone therapy). Has developed sxs of chest tightness/cough/wheezing/rhinorrhea in response to foods and various odors. Pulm function testing and methacholine challenge normal and chest sxs improved with a short course of Adviar and escalation of prednisone. Serum tryptase and w/u for mast cell activation syndrome has been unremarkable. She is maintaining her weight.
-She is getting endocrine consult (r/o adrenal insufficiency) and a second local I have recommended a trial of longacting oral antihistamines.

-What do you think about a trial of systemic Cromolyn?

-Any recommendations as far as lab work up for the hypersensitivity sxs she has developed recently?

A:

Thank you for your inquiry.

In my opinion, your patient has been evaluated thoroughly, and I personally have nothing directly to add or offer. Of course, cromolyn could be tried. It is almost devoid of side effects and therefore a trial would offer no harm, but it is unlikely that it would be effective.

Since I can offer you nothing substantial in regards to the further workup or management of this very challenging patient, I am asking for help from Dr. Gerald Gleich. Dr. Gleich is an internationally known expert in eosinophilic disease and hypereosinophilic syndrome. Due to the comprehensive evaluation that has been performed, he may have nothing to offer, but I will solicit his help nonetheless. When we hear from Dr. Gleich, we will forward his response to you.

Thank you again for your inquiry.

Sincerely,
Phil Lieberman, M.D.

We received a response from Dr. Gerald Gleich. Thank you again for your inquiry and we hope this response is helpful to you.

Sincerely,
Phil Lieberman, M.D.

Response from Dr. Gerald Gleich:
I suggest measurement of stool alpha-1- anti-trypsin as a measure of intestinal inflammation. She also needs a retrial of an elemental diet to determine if food ingestion results in remission of symptoms and eosinophilia; there are several varieties of elemental diet formulations and I suspect that with further testing one that is palatable could be found. Were biopsies of the gut performed? I did not see them listed. If biopsies were to be performed, it would be best that the patient be on an un-restricted diet for a time beforehand. A trial of Gastrochome should be performed.

To summarize: 1. The diagnosis is a clinical diagnosis presently and a tissue diagnosis would be desirable. See suggestion about feeding beforehand. 2. Treatment is identified above (elemental diet-this is both a diagnostic and therapeutic endeavor-and should be strongly advised). Gastrochrome is very safe and myself and my colleagues are using it extensively.

Hope these comments are helpful.

Gerald J. Gleich, M.D.
Professor of Dermatology and Medicine
Department of Dermatology
University of Utah

AAAAI - American Academy of Allergy Asthma & Immunology