Churg-Strauss Syndrome (CSS) consists of necrotizing vasculitis of small to medium sized arteries, necrotizing extravascular granulomas, and prominent tissue eosinophilia. CSS classically begins with a prolonged period of allergic rhinitis, nasal polyposis, and asthma. This is followed by the development of peripheral blood eosinophilia and eosinophilic tissue infiltrates, with culmination in a systemic vasculitic phase. Many published series investigating CSS exclude patients without asthma. Clinicians may overlook the diagnosis of CSS in patients who lack asthma.

We report the case of a 57-year-old male in whom CSS was diagnosed in the absence of asthma or bronchial hyperresponsiveness.

The patient had a nine-month history of progressive lower extremity weakness and pain accompanied by a peripheral eosinophilia (absolute eosinophil count approximately 5500 cells per microliter). Past medical history was significant for interstitial lung disease of unknown etiology, with a restrictive pattern on pulmonary function testing. A methacholine challenge test was negative. Definitive diagnosis of CSS was made with muscle and nerve biopsies, which demonstrated necrotizing vasculitis of medium-sized arteries, predominately lymphocytic extravascular granulomas with few eosinophils, and eosinophil-rich endomysial inflammatory infiltrate with evidence of eosinophil degranulation. The combination of clinical and laboratory features in this case meets four of the six 1990 ACR diagnostic criteria for CSS, confirming the diagnosis with greater than 99% specificity.

Churg-Strauss Syndrome does occur in the absence of asthma. Clinicians should not exclude the possibility of this diagnosis in patients lacking asthma.