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?