TRACHEOBRONCHIAL INVOLVEMENT IN WEGENERS GRANULOMATOSIS

This study was designed to characterize the clinical spectrum and cour se of tracheobronchial involvement in Wegener's granulomatosis (WG). O f the 51 patients with biopsy-proven WG who underwent bronchoscopy at least once at our institution between January 1982 and November 1993, 30 (59%) had endobronchial abnormalities due to WG. Initial findings i ncluded subglottic stenosis in five (17%), ulcerating tracheobronchiti s with or without inflammatory pseudotumors in 18 (60%), tracheal or b ronchial stenosis without inflammation in four (13%), and hemorrhage w ithout identifiable source in two (4%) patients. Nine patients with ul cerating tracheobronchitis on initial study had subsequent bronchoscop ies for continued symptoms, which in seven cases documented the progre ssion from ulcerating tracheobronchitis to stenosis without inflammati on. Bronchoscopic interventions included dilation by rigid bronchoscop e in three, YAG-laser treatment in one, and placement of silastic airw ay stents in three patients. Only the stents provided persistent airwa y patency Endobronchial biopsies were performed on 21 occasions in 17 patients. Half of the specimens were helpful in establishing the diagn osis and in all but three in assessing disease activity. While antineu trophil cytoplasmic antibody titers reflect overall disease activity, no correlation with endobronchial inflammatory activity was apparent.