A 47-year-old man with joint pain, cough, expectorating blood - tinged sputum, and with decreasing renal function was diagnosed to have Wegener's granulomatosis. The disease responded initially to therapy with alkylating cytotoxic agents by temporary disappearance of lung granulomas and marked remission of renal symptomatology.
Multiple relapses in the ensuing years were less sensitive to immunosuppression. After a course of 13 years, the patient developed the superior vena cava syndrome believed to be due to pressure of perivascular fibrotic mass spreading around the large arteries close the base of the heart.
The patient died 14 years after the onset of the disease from respiratory failure due to granulomatous vasculitis