Wegener's Granulomatosis of Lung: Diagnosis and Treatment

Twelve patients with pulmonary Wegener's granulomatosis are reported. In seven patients the disease appeared to be limited to the lungs; four patients had upper respiratory tract involvement, and one had cutaneous and subcutaneous lesions. A link between pulmonary and disseminated forms of this syndrome was provided by a patient who had a solitary pulmonary lesion for 4 years, after which involvement of the upper respiratory tract and the other lung developed. The course of the disease was extremely variable, rapid progression occurring in three patients, whereas one patient has had the disease for 12 years with little deterioration of health. Corticoid treatment resulted in transitory improvement in three patients and progression in four. Eight patients received cytotoxic therapy. One death occurred as the result of drug toxicity; at necropsy no evidence of Wegener's granulomatosis remained. Excellent responses have been obtained in three patients treated with chlorambucil and four treated with azathioprine. Surgical resection of pulmonary lesions was apparently curative in two cases but was unsuccessful in three. Recognition of this not uncommon disorder is important since cytotoxic therapy appears to be uniformly effective.