The Churg Strauss Syndrome

During the first half of this century, several authors reported a variant of a small vessel vasculitis that appeared similar to the then recognized polyarteritis nodosa. The condition was distinguished by a significant atopic component, in the form of allergic rhinitis, bronchial asthma, and hypereosinophilia (1). Such patients frequently had a poor prognosis, with many patients dying as a result of cardiac complications, often within months of diagnosis. Although it has previously been called by several names including allergic angiitis and granulomatosis, it is now most commonly referred to as the Churg Strauss syndrome (CSS). Renal involvement in this condition is frequent although usually mild; however, an increasing number of case reports attest to the potential for severe renal disease to occasionally develop. Several lines of evidence suggest that CSS is not simply the coincidental development of vasculitis in patients with asthma. The asthma seen in CSS is notable for being of late onset; it is frequently severe and is associated with a greater degree of eosinophilia than is typically seen in bronchial asthma. There is often no personal or family history of atopic disease (2). In addition, cases are well described in which the asthma develops shortly before or simultaneously with vasculitis (3,4). The recognition of CSS as a separate disease entity is important, because its distinctive natural history (2,5), its frequent rapid response to treatment, and its good overall prognosis (4) suggest pathogenic mechanisms that differ either in nature or in degree compared with other forms of necrotizing vasculitis.