Atypical presentation of Churg-Strauss syndrome: another "forme fruste" of the disease?

Abstract

Vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vessels. A broad and heterogenous group of syndromes may result from this process, because any type, size, and location of blood vessel may be involved. The cause of these conditions remains unclear, but an autoimmune inflammatory process, characterized by involvement of both neutrophils and endothelial cells, seems to play an important role. In 1951, Churg and Strauss described a clinical syndrome of severe asthma, hypereosinophilia with eosinophilic infiltrates, eosinophilic vasculitis, and granulomata in various organs. Asthma may precede this vasculitis by many years. We report a case of anti-neutrophil cytoplasmic antibody-positive, pauci-immune, crescentic, necrotizing glomerulonephritis with peripheral and interstitial eosinophilia but without asthma. This is very unusual in Churg-Strauss syndrome.