Cases of Wegener's granulomatosis with exclusive renal involvement are
very unusual. Here we report a patient who presented which acute rena
l failure with massive extracapillary proliferation on renal biopsy, w
ithout extrarenal manifestations. After 9 years on chronic hemodialysi
s she developed several otorhinological abnormalities and finally pulm
onary nodes. Lung biopsy showed a granulomatous vasculitis characteris
tic of Wegener's granulomatosis. Wegener's disease can recur after pro
longed asymptomatic periods. In those cases with exclusive renal invol
vement, diagnosis can be delayed until the appearance of respiratory m
anifestations.