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44 - CHURG-STRAUSS SYNDROME

from PART V: - SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM

Published online by Cambridge University Press:  06 January 2010

Louis R. Caplan
Affiliation:
Beth Israel Deaconess Medical Center, Boston
Julien Bogousslavsky
Affiliation:
Valmont Clinique, Glion, Switzerland
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Summary

The Churg-Strauss syndrome (CSS) is a type of small-vessel vasculitis that involves the capillaries, arterioles, and venules. There are different clinical and histopathological criteria for the diagnosis of CSS. The clinical criteria of J. G. Lanham and his group for the diagnosis of CSS is a triad consisting of asthma, peak eosinophilia > 1.5 x 109/L, and systemic vasculitis involving two or more extrapulmonary organs. Glucocorticoids remain the main stay of therapy in CSS. Initial therapy with prednisone is usually pulsed (15 mg/kg over 60 minutes repeated at 24-hour intervals for 1-3 days) followed by prednisone 1 mg/kg per day. Involvement of the central nervous system (CNS) is associated with an increased risk for mortality and requires aggressive treatment with steroids and immunosuppressants. The condition should be considered in any patient who has asthma and develops eosinophilia and peripheral and/or CNS signs.
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Publisher: Cambridge University Press
Print publication year: 2008

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