Diagnostic and therapeutic aspects of the surgical approach to Wegener's granulomatosis.

Over a 16 year period, 47 patients with Wegener's granulomatosis were treated at the National Institutes of Health. Age at the onset of disease ranged from 15 to 75 years with a mean of 42.2 years. The group was evenly divided between male and female subjects. With seven exceptions, all patients had the classic form of Wegener's granulomatosis, with upper airway and pulmonary disease and glomerulitis. The most consistently abnormal laboratory test was an elevated erythrocyte sedimentation rate. Most of the patients were treated with cyclophosphamide alone or in combination with prednisone. In 37 patients the disease now is well controlled or in complete remission from 1 to 9 years after the onset of symptoms. Of the 10 patients who died, only two were considered treatment failures. Forty-one of the 47 patients had chest x-ray changes at some point in their clinical course. Nineteen had bilateral involvement and 22 unilateral disease. Three of 11 patients with nodular disease had cavitation, and four of 21 with less discrete infiltrative disease demonstrated cavitation. Four patients had an associated pleural effusion and another four had pleural thickening. As part of the diagnostic procedure, 24 patients underwent an open lung biopsy. Two patients had a lobectomy for presumed neoplastic disease. In one patient the diagnosis of Wegener's granulomatosis was made by endobronchial biopsy. In the remaining patients the diagnosis was made by biopsy of another site. Endobronchial involvement is being seen with the increasing patient survival. Three patients had subglottic tracheal stenosis necessitating tracheal dilatations. One of the three also had multiple areas of bronchial stenosis responding to cyclophosphamide therapy. A fourth patient had progressive left main-stem bronchial stenosis which necessitated a sleeve resection. Five patients had pericarditis secondary to Wegener's granulomatosis.