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Idiopathic Hypereosinophilic Syndrome &
Churg Strauss Syndrome
Hypereosinophilic Syndrome (HES) is a group of disorders where very high numbers of eosinophils are found in peripheral blood counts (blood test) and organ tissue damage occurs. Unlike eosinophilic gastrointestinal disorders (EGID), which only affects the digestive tract, HES may affect any organ in the body. To diagnose HES, more than 1500 eosinophils/microliters must be found in the blood for more than six months with injury (damage) to organs. Chronic eosinophilic leukemia (CEL) means the eosinophils are clonal (all from the same cell line, identical).
For additional resources on HES www.HESResource.com
Churg Strauss Syndrome (CSS) is an autoimmune disorder where the small blood vessels are the primary target (vasculitis) of the eosinophils. In CSS, individuals typically have lung involvement, asthma, and may also have involvement of the heart, GI tract, brain and other organs. Eosinophils are involved in causing this vasculitis. Tests to diagnose CSS include a complete blood count, a chest x-ray, pulmonary (lung) function tests, a biopsy and other tests based on individual symptoms. Your physician will determine the necessary tests to make the diagnosis.
Tests to diagnose HES include a complete blood count and examination of other organs based on symptoms or other abnormal test results. Additional tests usually include a complete blood count and eosinophil count, blood samples for liver and kidney function, a blood test for Vitamin B12, erythrocyte sedimentation rate (general sign of inflammation), and a blood test for tryptase. Depending on symptoms and test results, other studies may be performed. For instance, ultrasound (echocardiography) is used to look at the function of the heart. A chest x-ray may be done to examine the lungs. As always, your medical team can best guide the necessary diagnostic tests on an individual basis.
Idiopathic Hypereosinophilic Syndrome
Criteria for diagnosis include:
1. Peripheral blood eosinophilia (high numbers of eosinophils in the blood) more than 1500 eosinophils/ , for at least six months’ duration.
2. End-organ (heart, lungs, GI tract, brain, skin, etc) involvement with eosinophil tissue infiltration (invasion) and injury.
3. No other known causes for the eosinophilia (e.g. parasitic infections).
High numbers of eosinophils in blood can be caused by many different problems, including EGID, and doesn't necessarily mean there is HES. Many children with EGE have higher than usual peripheral blood eosinophils but it does not mean that they have, or will develop HES.
Churg Strauss Syndrome (CSS)
Criteria for diagnosis include:
1. Asthma.
2. Eosinophilia (high levels of eosinophils in the blood, >10% on differential white blood cell count.
3. Mononeuropathy (inflammation or injury of the nerves);
4. Transient (not permanent) pulmonary infiltrates on chest X-rays. The chest x-ray will be abnormal.
5. Sinus involvement.
6. Biopsy containing a blood vessel with eosinophils (vasculitis).
You can find additional information on CSS on the following sites:
Churg Strauss Syndrome Association
The Johns Hopkins Vasculitis Center
Churg-Strauss Syndrome International Support Group
Treatment of Hypereosinophilic Syndromes
Treatment of hypereosinophilic syndromes (HES) and chronic eosinophilic leukemia (CEL).
In the hypereosinophilic syndromes, high numbers of eosinophils are found in the blood and affect multiple organs in the body. This may include the stomach and intestines, the heart, lungs, skin and others. The eosinophils cause inflammation and eventually damage to the involved organs.
Higher dose systemic (oral) steroids are often needed to control HES with organ involvement. Steroids are medications that fight (suppress) many types of inflammation. They are not specific for suppressing eosinophils, although eosinophils are particularly sensitive to them. Steroids can be taken intravenously (IV), or ingested orally. Systemic steroids, those that are absorbed into the bloodstream (oral or IV), are very effective for treating a number of eosinophilic disorders. Unfortunately, the disease may return when the steroids are stopped. Steroids given in this manner may have many harmful side effects when used for long periods of time. Serious side effects can include osteoporosis (brittle bones from bone loss), infections, adrenal insufficiency (body becomes unable to properly respond to illness or stress), avascular necrosis (collapse of the bones in a joint, usually the hip), and stunted growth. Common side effects may include fluid retention (swelling), increased appetite, “moon-face”, and irritability.
was developed to treat certain types of leukemia. Imatinib may induce remission in select types of HES. Not all patients with HES will respond to Imatinib. Genetic testing (for FIP1L1-PDGFRα gene rearrangement) can help determine if Gleevec is likely to help.
The calcineurin inhibitors include cyclosporine (Neoral®, Sandimmune®, Gengraf®) and tacrolimus (Prograf®). These are very potent medications that suppress the immune system by interfering with the function of T cells. They are used primarily to prevent organ rejection in people who have had organ transplants. They may also be of benefit in some patients with the hypereosinophilic syndrome. Because calcineurin inhibitors have a number of potentially harmful side effects, they are reserved for more severe and refractory (treatment-resistant) cases. Side effects include kidney failure, nerve damage, headaches, hair loss or excess growth, elevated cholesterol, high blood pressure, diabetes and development of cancer. Blood levels of these medications must be carefully monitored. Many other medications, particularly antibiotics, can affect the blood levels.
Agents used to treat cancers are not specific for eosinophilic disorders, but may be helpful in some types of HES. These are potent medications with potentially harmful side effects and are reserved for more severe disease. Careful monitoring while taking these medications is very important.
Methotrexate
Hydroxyurea
Cyclophosphamide
etoposide
Vincristine
Bone marrow transplant
Details of these potent medications are beyond the scope of this review. Further information can be found at .
Alpha- Interferon is used for a variety of diseases, including leukemia. It is given by injection (either in the muscle or under the skin). Interferon may have many side effects and requires careful monitoring.
Therapy for hypereosinophilic syndrome requires careful discussion with your health care providers regarding the risks and benefits of the treatment for your specific HES- related organ involvement.
Treatment of Churg Strauss Syndrome (CSS)
CSS is an autoimmune disorder where the small blood vessels are the primary target (vasculitis). In CSS, individuals typically have lung involvement, asthma, and may also have involvement of the heart, GI tract, brain and other organs. Eosinophils are involved in causing this vasculitis. Treatment depends on the severity of the disease and organ involvement. More severe disease may require more aggressive therapy.
are the primary treatment for CSS. Glucocorticoids (“Steroids”) are medications that fight (suppress) many types of inflammation. Please see the section above on treatment of HES for more detailed information on steroids.
agents may be used when steroids are not working to adequately control the disease. These are all very potent therapies that increase the risk of infection and have serious potential side – effects.
• Cyclophosphamide
• Mycophenolate mofetil
• Azathioprine
• Methotrexate
• TNF-alpha blocking agents (etanercept or infliximib)
• Intravenous immunoglobulin (IVIG)
Your doctor can provide information on these powerful medications
More information can also be found at .
Prognosis
The prognosis in HES and CSS depends on the organ systems involved, disease severity and response to therapy. Outcomes can vary greatly from one person to the next. Your doctor can best answers questions about prognosis in HES and CSS on an individual basis.
Churg Strauss Syndrome Association
The Johns Hopkins Vasculitis Center
Churg-Strauss Syndrome International Support Group
emedicine.com (Hypereosinophilic Syndrome)
Future Directions
Therapies in development for treatment of eosinophilic disorders are directed at reducing the production or stimuli that attract the eosinophils. These include:
•
IL(interleukin)-5 inhibitors
• Anti-eotaxin
• CCR3 (chemokine receptor) antagonists
Click here for links to .
References
Straumann A, Simon HU. The physiological and pathophysiological roles of eosinophils in the gastrointestinal tract. Allergy 2004;59:15-25
Coutre S, Gotlib J. Targeted treatment of hypereosinophilic syndromes and chronic eosinophilic leukemias with Imatinib mesylate. Semin Cancer Biol. 2004 Feb;14(1):23-31.
Roufosse F, Cogan E, Goldman M. Recent advances in pathogenesis and management of hypereosinophilic syndromes. Allergy. 2004 Jul;59(7):673-89.
Lin DA, Boyce JA The idiopathic hypereosinophilic syndrome. Allergy Asthma Proc. 2003 Nov-Dec;24(6):417-20.
Garrett JK, Jameson SC, Thomson B, Collins MH, Wagoner LE, Freese DK, Beck LA, Boyce JA, Filipovich AH, Villanueva JM, Sutton SA, Assa'ad AH, Rothenberg ME. Anti-interleukin-5 (mepolizumab) therapy for hypereosinophilic syndromes. J Allergy Clin Immunol. 2004 Jan;113(1):115-9.
Berki T, David M, Bone B, et al. New diagnostic tool for differentiation of idiopathic hypereosinophilic syndrome (HES) and secondary eosinophilic states. Pathol Oncol Res 2001;7(4):292-7
Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. 2003 Nov-Dec;21(6 Suppl 32):S69-77. Review.
Hellmich B, Gross WL. Recent progress in the pharmacotherapy of Churg-Strauss syndrome. Expert Opin Pharmacother. 2004 Jan;5(1):25-35. Review.
Abril A, Calamia KT, Cohen MD. The Churg Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum. 2003 Oct;33(2):106-14. Review.
Noth I, Strek ME, Leff AR Churg-Strauss syndrome. Lancet. 2003 Feb 15;361(9357):587-94. Review.
All information contained within the American Partnership for Eosinophilic Disorders’ website is intended for educational purposes only. Visitors are encouraged to consult other sources and confirm the information contained within this site. Consumers should never disregard medical advice or delay in seeking it because of something they may have read on this website.
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