Significant eosinophilia is a prominent feature in Churg-Strauss syndrome b
ut has only rarely been described in Wegener's granulomatosis (WG). We desc
ribe two Wegener's granulomatosis patients with > 30% eosinophilia on their
initial presentations, Other etiologies that could account for their eosin
ophilia were excluded. Both patients had pulmonary alveolar hemorrhage, sin
usitis, arthritis, high-titer cytoplasmic antineutrophil cytoplasmic antibo
dies (cANCA), and proteinase-3 antibodies, but no evidence of renal disease
. Herein we discuss eosinophilia, the differential diagnosis of pulmonary i
nfiltrates and eosinophilia, the role of cANCA in vasculitis and autoimmune
disease, compare Wegener's granulomatosis and Churg-Strauss syndrome, and
review possible pathogenic mechanisms.