We report clinical and pathologic findings in 22 patients with Wegener's granulomatosis collected from 1966 to 1989. Ten cases were analyzed retrospectively. Organs affected included the lungs (n = 18), upper airways (16), kidneys (15), musculo-skeletal system (10), eyes (8), skin (7) and ear (5). Clinical manifestations of airway involvement included nasal obstruction, dysphonia and epistaxis. Lung involvement was evident in chest X-rays in 18 patients, 14 with a nodular aspects. Histologic study in 14 of these patients showed necrotizing and granulomatous vasculitis. Clinical evidence of nephropathy was evident in 15 patients and led to rapidly progressive renal failure in 8. Biopsy in this group (n = 14) revealed focal glomerulonephritis in 6 and diffuse disease in 8. Overall, 13 patients died: 5 without diagnosis, 4 from renal failure, 2 from sepsis, 1 from a lymphoma developing 3 years after immune suppressive therapy and 1 from unknown causes. Among survivors, one received a renal transplant and one remains in chronic dialysis. The diagnosis of Wegener's granulomatosis is therefore based on clinical findings including rhino pharyngeal, pulmonary and renal manifestations.