biopsies for eosinophilic gastritis

[CINDY4]

My 10 yr old daughter has what drs believe is Churg-Strauss a rare vasculitis.  She has had many sugeries (8) and countless biopsies since finding lung disease on a CT 2 yrs ago.  She has biospy proven eosinophilic infiltrates in her lungs and sinus.  Her lungs are the main target of this autoimmune disease, but it is systemic and she has problems in her nerves and GI as well.  She has had extensive GI problems for about 3 years, which started when she was put on daily steroids (10mg).  Once she started steroids stomach pain was constant.  Her dr had her on 80 mg of Prilosec and reglan daily, but pain persisted.  About 9 months after starting the steroids for what was then thought to be bad asthma, the pain was uncontrollable.  At that time a scope showed multiple bleeding ulcers.  She was put on Carafate and also had a nisson.  The steroids were weaned and the pain was much better.  The reflux never came back but lingering abdominal pain continued.  

Six months after the nisson, the lung disease was found and a biopsy showed she had eosinophilic pneumonia.  Because of the bleeding ulcers which were thought to be from the steroids, she was put on 2500mg of IV solumedrol monthly.  She had no stomach pain on this med but around the time for another infusion the pain would start up again. We eventually found this was a systemic autoimmune disease, and a diagnosis of Churg-Strauss was entertained.  Drs then thought that maybe the bleeding ulcers she previously had were really eosinophilic gastroentritis.  New scopes were(colon and upper GI) done which biopsies showed patchy chronic inflammation but no eosinophils( this was 4 weeks after getting 2500mg solumedrol, and on 5mg daily pred). The IV steroids were stopped and 10 mg daily pred was started once lung functions stabilized.  Stomach pain continued and a hidascan showed bad gallbladder.  So out came the gallbladder, which also showed chronic inflammation.  Pain did get better  but returned again in a couple of months.

Problems with the lungs got worse and steroids were then started again at 60 mg daily weaning over 3 weeks to 10 mg daily.  Her stomach pain has continued and got severe again at Christmas, another scope was done fearing she had bleeding ulcers but she didn't and biopsies again showed inflammation but no eosinophils. GI dr feels this is eosinophilic gastritis and steroids have just supressed the eosinophils on biopsy.  He has said a full thickness biopsy is sometimes what it takes for diagnosis.  Do you think there is enough evidence here for diagnosis without anymore surgeries or scopes( he also mentioned weaning steroids and repeat scope)?  What meds would be used besides steroids?  GI has mentioned low dose chemo.  Since she has been on so many steroids, would it seem steroids are not enough?  What are your experiences with this disease?  Thank you!  Cindy

[Kevin Pho, MD]

This is certainly a difficult case, and one that is likely out of my range of expertise.  

The diagnosis of esosinophilic gastritis is typically confirmed by endoscopic biopsies which reveals more than 20 eosinophils per high power field on microscopic examination.  Upper endoscopy with biopsy of the stomach and small intestine is diagnostic in at least 80 percent of patients.  Multiple biopsy samples should be taken from both the stomach and small intestine because of the patchy nature of the disease.  

Without being personally involved in the case, I cannot comment on whether there is enough evidence to advise against a full-thickness biopsy.  

There have been no prospective, randomized clinical trials regarding therapy.  Here are some investigational options that you may want to consider.

1) Oral cromolyn (800 mg/day in four divided doses) has been effective for short- and long-term management.

2) Ketotifen (Zaditen), a mast cell stabilizing agent, has been helpful in case reports. The drug is approved for treatment of urticaria in Canada, Europe, and Japan, but is not available in the United States.

3) The leukotriene antagonist, montelukast, was effective in some case reports but not in others.

4) A clinical response to suplatast tosilate (a novel antiallergic drug that suppresses cytokine production including IL-4 and IL-5 from Th2 cells) was described in a single patient.

5) An ongoing study at the National Institutes of Health in the United States is evaluating the efficacy and safety of a humanized anti interleukin-5 antibody in patients refractory to other treatments.  In a preliminary report involving four patients, treatment reduced peripheral and tissue eosinophil counts but had no effect on symptoms.

Followup with your personal physician is essential.

This answer is not intended as and does not substitute for medical advice - the information presented is for patient education only. Please see your personal physician for further evaluation of your individual case.

Thanks,
Kevin, M.D.

Bibliography:
Wallace et al.  Eosinophilic gastroenteritis.  UptoDate, 2004.

[CINDY4]

Forgot to mention her symptoms are lower abdominal pain( navel and below), almost daily diarhea (diarrhea), very few normal stools, nausea, motion sickness, pain after she eats.  Every now and then she will have a good day and not have many problems, other days pain is severe.  Most days she is somewhere between the two, a moderate level of pain.  Also 3 weeks ago drs swithched the prednisone 10 mg every other day to Entocort 9mg daily in hopes of helping GI pain.  Has not worked, and her main dr an A/I feels these problems are not related to steroid side effects.

[CINDY4]

Sorry, forgot one other thing.  At the time this last scope was done a sonogram and upper GI with small bowel follow through was also done.  Sonogram showed mildly enlarged spleen, and some free fluid in her abdomin (abdomen).  Upper GI was normal was borderline delayed motility. Also blood counts at this time have normalized with the exception of ECP (eosinophilic cationic protein), this is a newer test that shows the activated eosinophils.  This count stays between 40-50 with normal being 0-24.  Her eos count are now normal on steroids, but are around 15% after steroids are weaned.

[janntom]

A related discussion, steroids was started.