Delayed form of Wegener's granulomatosis

We have described a patient in whom renal manifestations preceded the pulmonary findings of Wegener's granulomatosis by two years. He presented with rapidly progressive glomerulonephritis requiring chronic hemodialysis despite treatment with prednisone and azathioprine. Wegener's granulomatosis was diagnosed only after other signs of the generalized form of the disease had become manifest. Review of the literature revealed four similar cases. In patients with similar clinical presentations, serious consideration should be given to this delayed form of the syndrome. A course of cytotoxic therapy should be tried while the patient is followed up for further manifestations of generalized Wegener's granulomatosis.