Treatment of Wegener's granulomatosis of lung

Thirteen patients with Wegener's granulomatosis have been treated with cytotoxic agents. Only one died from the disease whereas two died of infectious complications of therapy and one of heart disease. Seven of the nine survivors are well without medication; one is alive with renal insufficiency and one is in the 5th year of treatment. Chlorambucil was least toxic and should be tried first. Cyclophosphamide was more effective than chlorambucil or azathioprine but, because of side effects, should rarely be used initially. Differentiation of Wegener's granulomatosis from lymphomatoid granulomatosis, which it resembles clinically, roentgenologically and pathologically, is important since the latter disease responds differently to cytotoxic drug therapy.