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Treatment of CSS

TREATMENT AND PROGNOSIS OF CSS

Systemic corticosteroids are the mainstay of treatment for patients with CSS. Prednisone is used to treat asthma and intravenous steroids may be required to handle acute flares. Steroids are required for months, if not years, and some patients are unable to come off of this therapy due to recrudescence of underlying symptoms. When the heart, GI tract and nervous system are involved, the chemotherapeutic agent cyclophosphamide is added on to suppress the systemic eosinophilic effects. Unfortunately, both of these therapies are associated with significant side effects, and the goal of the treating physician is to try to minimize disease pathogenesis while minimizing therapy side effects, which often rival CSS manifestations. This is difficult to do so other agents are often added, including azathioprine, interferon alpha, mycophenolate and methotrexate, with mixed results.

A minority of patients with CSS may come off of systemic corticosteroid therapy completely and live, long healthy lives. Most patients however, require systemic steroids continuously and efforts to reduce doses are associated with significant relapse of symptoms. Involvement of the heart, kidney and GI tract dramatically increases mortality, and 50% of those who are left untreated may die from the disease. Others develop infectious complications from the constant immunosuppression.

FUTURE DIRECTIONS

The etiology of CSS remains a mystery and a lot of research is being performed to try to understand the underlying pathophysiology and genetics of this disease. Specifically, CSS subjects are being studies to assess whether or not there are specific genes that predispose these individuals to this syndrome; it is hoped that this may lead to a better understanding of not just CSS, but also asthma, and that new therapies may be developed to treat this difficult syndrome.

For more information on Churg Strauss syndrome:
Churg Strauss Syndrome Association
The Johns Hopkins Vasculitis Center
Learn More: Clinical Trials

References
Diagnosis & Treatment of Churg Strauss Syndrome (CSS)
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.
About the AUTHOR
Dr. Michael Wechsler is a specialist in Pulmonary and Critical Care at Brigham and Women’s Hospital in Boston, MA and is on the faculty of Harvard Medical School. He is on the Steering Committee of the NIH’s Asthma Clinical Research Network and is active in clinical research involving both asthma and Churg-Strauss syndrome.

© American Partnership for Eosinophilic Disorders (APFED) 2008, 2009, 2010, 2011. All rights reserved.
Updated 3-11-2011

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BIOGRAPHY OF AUTHOR
Dr. Michael Wechsler is a specialist in Pulmonary and Critical Care at Brigham and Women’s Hospital in Boston, MA and is on the faculty of Harvard Medical School. He is on the Steering Committee of the NIH’s Asthma Clinical Research Network and is active in clinical research involving both asthma and Churg-Strauss syndrome.