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Avatar universal

allergies and Churg-Strauss vasculitis

Hello,

Thank you for taking the time to read this.

I am a 29-year-old female recently diagnosed with Addison's Disease, hypothyroidism, pancreatitis, possible hypoparathyroidism and ovarian failure, and allergies to over 40 foods. I have not been tested for environmental allergies yet. My doctors believe there is a vascular component underlying all of these endocrine problems, and have collectively suggested Churg-Strauss vasculitis. This is because I have had several instances of very pronounced eosinophilia (blood and tissue biopsy) over the last couple of years, weight loss, unexplained neuropathies, intermittent episodes of lower GI bleeding, intermittent pancreatitis and gall bladder dysfunction, rashes, very high fevers, joint and muscle pain, etc.

For some reason, I do not have antibodies to anything. However, my doctors assume that my problems are autoimmune. I do have an enlarged thymus.

I have yet to see a rheumatologist. My symptoms seem to flare together and are definitely more pronounced lately, with the high pollen counts, and also in the heat. I have never been diagnosed with asthma, which I understand is usually a big component in CSS. However, my food allergies were adult-onset, and beginning just last year (at this time), I had what I suppose were asthma attacks outdoors - trouble breathing, feeling of being "squeezed", would resolve when coming indoors.

My question: If I do in fact have CSS, can it be controlled just by eliminating and/or being treated for specific allergies (triggers), or is it always necessary to treat with steroids and either chemo or immunosuppressive drugs? I am currently unable to function without at least 20-40mgs of Prednisone/day.

Also, could the thymus itself be causing these problems? At what point do doctors consider removing it?

Thank you in advance!
2 Responses
242588 tn?1224275300
MEDICAL PROFESSIONAL
Many features of your illness are consistent with the diagnosis of the Churg-Strauss Syndrome, a systemic vasculitis.  There is evidence to suggest a close relationship, or at least a clinical overlap, between this disease and a number of hypereosinophilic disorders (see below).  While polyendocrinopathies can theoretically be on the basis of systemic vasculitis, with involvement of individual endocrine glands, another perhaps more likely explanation would be pituitary gland failure, panhypopituitarism, either secondary to vasculitis or to a non-vasculitis cause, for example a pituitary tumor.

The abstract, below addresses your question of treatment of Churg-Strauss.  There is no good evidence to suggest that this disorder might respond to the treatment or avoidance of specific allergens.  

That you do not “have antibodies to anything” is likely to be associated with the thymus enlargement.  Sorting this out would best be done by a rheumatologist or an immunologist, and in the absence of multi-expert opinion  favoring the removal of your thymus gland, I would be most reluctant to proceed with that, for fear that the removal might further complicate your already very complicated medical problems.

Speaking of expert opinion, you would do well to consult with knowledgeable physicians with a special interest in vascular diseases, including CSS, and in hypereosinophilic states; physicians with both clinical experience and  understanding of the basic science involved.  Your doctors would almost certainly be able to identify such individuals or research institutions.

Good luck,

ABSTRACT:
Title: Hypereosinophilic syndrome and Churg-Strauss syndrome: is it clinically relevant to differentiate these syndromes?
Authors: Hellmich, B. Holl-Ulrich, K. Merz, H. Gross, W L.
Institution: Medizinische Klinik, Kreiskrankenhaus, Am Aussichtsturm 5, 73207, Plochingen
Source: Internist. 49(3):286-96, 2008 Mar.
Contact: b.hellmich@kk-es.de

Churg-Strauss syndrome must be distinguished from a group of hypereosinophilic disorders: Löffler's syndrome, the hypereosinophil syndrome, eosinophilic gastroenteritis, chronic eosinophilic pneumonia, and eosinophilic leukemia. The two phenomena of leukotriene antagonist use and the occurrence of Churg-Strauss syndrome are probably related more to the reduction in corticosteroid dose afforded by leukotriene antagonists (and the resultant unmasking of latent Churg-Strauss syndrome) than to any truly causal relationship. Patients with mild disease may be treated with prednisone. Those with evidence of neurologic, cardiac, renal, or gastrointestinal involvement should be treated with cyclophosphamide in addition to corticosteroids. Although clinical remissions may be obtained in more than 90% of patients with Churg-Strauss syndrome, disease recurrences are seen in 25%. In most cases, relapses are heralded by the return of eosinophilia. Approximately 50% of cases of Churg-Strauss syndrome are associated with ANCA, usually MPO-ANCA, but the percentage may be higher among untreated patients.

Churg-Strauss syndrome and the hypereosinophilic syndrome share many clinical features, particularly in the early disease stages. Beside blood and tissue eosinophilia, peripheral neuropathies, cutaneous manifestations, eosinophilic alveolitis and gastroenteritis are frequently found. In contrast to the hypereosinophilic syndrome, Churg-Strauss syndrome is defined by the presence of systemic vasculitis. However, frequently symptoms related to eosinophilia are (mis)interpreted as indirect signs of vasculitis. New treatment modalities and diagnostic methods render the early differentiation between Churg-Strauss syndrome and the hypereosinophilic syndrome increasingly clinically important. Patients with hypereosinophilic syndrome should be tested for the presence of the FIP1L1-PDGRFA-mutatition in order to identify patients that could benefit from a treatment with a tyrosine kinase inhibitor such as Imatinib. At present, immunosuppression is still the treatment of first choice for Churg-Strauss syndrome. Novel treatment modalities for both diseases include immunomodulation with interferon alpha and biologics such as antibodies against interleukin 5.
Avatar universal
Thank you very, very much for your quick and detailed reply! I really appreciate this information and will share it with my doctors.

I had an MRI of the pituitary a few months ago that did not show any evidence of a tumor, and also an MRA of the Circle of Willis that was normal. I don't know if you are able to answer any more questions here, and I don't mean to seem greedy. But, are there other imaging modalities or blood tests that should be considered for panhypopituitarism? How exactly is autoimmune hypopituitarism diagnosed?

I think my doctors assume my adrenal insufficiency is primary because I had the typical electrolyte/aldosterone abnormalities (and related symptoms) prior to treatment. So there hasn't been much discussion about the pituitary, although for the reasons you mentioned, perhaps it should be revisited.

I do wish there was a way of finding out what really triggered these problems, and eradicating that specifically, rather than treating symptomatically. But for now, gathering knowledge helps me feel as though I am maintaining some control of my situation. So thank you again for this information.

Take care,
Leigh
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