Pathogenesis

Although the etiology of CSS is unknown, eosinophils are thought to play a central role in tissue injury. Precipitating factors may include inhaled antigens that stimulate eosinophils. Histopathological findings are remarkable for the presence of eosinophilic infiltrates, granulomata, and, in the most typical biopsies, necrotizing vasculitis of small- to medium-sized vessels (Fig. 7) (3). There are many reports of CSS cases occurring in association with the use of leukotriene receptor antagonists and various inhaled GC, but a definite causative role for these agents in the pathogenesis of CSS has not been firmly established.

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