Tracheobronchial involvement in Wegener's granulomatosis.

Abstract

This study was designed to characterize the clinical spectrum and course of tracheobronchial involvement in Wegener's granulomatosis (WG). Of 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 included subglottic stenosis in five (17%), ulcerating tracheobronchitis with or without inflammatory pseudotumors in 18 (60%), tracheal or bronchial stenosis without inflammation in four (13%), and hemorrhage without identifiable source in two (4%) patients. Nine patients with ulcerating tracheobronchitis on initial study had subsequent bronchoscopies for continued symptoms, which in seven cases documented the progression from ulcerating tracheobronchitis to stenosis without inflammation. Bronchoscopic interventions included dilation by rigid bronchoscope in three, YAG-laser treatment in one, and placement of silastic airway stents in three patients. Only the stents provided persistent airway patency. Endobronchial biopsies were performed on 21 occasions in 17 patients. Half of the specimens were helpful in establishing the diagnosis and in all but three in assessing disease activity. While antineutrophil cytoplasmic antibody titers reflect overall disease activity, no correlation with endobronchial inflammatory activity was apparent.

Modern imaging of the tracheo-bronchial tree

“…Fortunately, the survival has been significantly improved with the instigation of combination therapies using cyclophosphamide and corticosteroids, with a mean five-year survival rate now of 90%-95% [63] and a mean survival rate of 21.7 years [64] . Airway involvement in WG is found in 15%-60% of patients but is rarely a presenting feature of the disease for most patients since laryngeal and trachea-bronchial involvements are usually late manifestations of the disease (Figure 18) [68][69][70][71][72][73][74][75] . Bronchoscopic findings include subglottic stenosis, ulcerating tracheobronchitis with or without inflammatory polyps or pseudotumors, and tracheal and bronchial stenosis [67,68] .…”

“…Airway involvement in WG is found in 15%-60% of patients but is rarely a presenting feature of the disease for most patients since laryngeal and trachea-bronchial involvements are usually late manifestations of the disease (Figure 18) [68][69][70][71][72][73][74][75] . Bronchoscopic findings include subglottic stenosis, ulcerating tracheobronchitis with or without inflammatory polyps or pseudotumors, and tracheal and bronchial stenosis [67,68] . Subglottic stenosis is the most frequent large airway manifestation of WG occurring in 8.5%-50% of patients [76,77] .…”

Comprometimento da árvore respiratória na granulomatose de Wegener

“…[4][5][6][7] In approximately 25% of the patients with WG, involvement of the airways can be the only manifestation of the disease.…”

“…2,3 The involvement of the airways is one of the major characteristics of WG, and occurs in 15%-55% of the patients. [4][5][6][7] The symptoms include cough, hemoptysis, stridor, sibilant rales, and dyspnea. 7,8 The manifestations of WG in the respiratory tract include nasal stenosis, nasal cartilage necrosis, subglottic stenosis, tracheal and bronchial stenosis, granulomatous nodules and masses, alveolar infi ltrates, and cavities.…”

The treatment of Wegener's granulomatosis

“…These may lead to recurrent obstructive pneumonias and/or impairment of ventilation significantly reducing a patient's activity. Our group will soon publish the results of our experience with silastic intraluminal stents both in the trachea and the major bronchi [32]. The initial evaluation is quite promising.…”