The Churg Strauss Syndrome (allergic granulomatous angiitis): review and update

Objectives: Review the clinical and physiopathologic aspects of the Churg-Strauss syndrome (CSS), including recent data regarding treatment and possible etiologic and triggering factors. Methods: A search of the Medline database was conducted between 1966 and 2002, regarding CSS and related vasculitic conditions. Original articles were reviewed as well as major vasculitis textbooks, which were also examined for original references. Results: CSS has been increasingly recognized during the past few decades, but remains an uncommon disease of unknown cause. The disorder had been traditionally classified as a variant of polyarteritis nodosa until its updated description by Churg and Strauss in 1951. Although it shares various clinical laboratory and pathologic characteristics with polyarteritis nodosa and Wegener granulomatosis, a distinct combination of features makes it a separate entity. The presence of asthma, usually of adult onset, along with other allergic symptoms, peripheral and tissue eosinophilia, and systemic vasculitis should prompt the clinician to consider the diagnosis, seek potential confirmation with a tissue biopsy, and begin therapy to minimize complications and prevent permanent organ damage. The treatment of CSS has been mainly extrapolated from other vasculitides, and the literature addressing drug therapy for this specific syndrome is limited. Conclusions: CSS is a distinct entity that should be recognized and distinguished from other forms of vasculitis to provide the appropriate early treatment, which could prevent permanent organ damage.