Subglottic stenosis in granulomatosis with polyangiitis (Wegener granulomatosis)

A 36 year old woman presented with a 1 year history of nasal obstruction, exertional dyspnea with biphasic stridor and 10 kg unintentional weight loss. Laboratory investigations revealed that her hemoglobin level was 8.1 g/ dL and erythrocyte sedimentation rate was 41 mmph. Her anti-neutrophil cytoplasmic antibodies were positive. Chest radiograph revealed a small faint patch over left upper lung. Sinoscopy revealed a roomy nasal cavity caused by atrophy of the mucosa, thick discharge, lots of dark yellow crusts, epistaxis and a large perforation of the nasal septum. These findings suggested atrophic rhinitis complicated with perforation of the nasal septum (FIGURE 1 arrows: perforation of the nasal septum, asterisk: atrophy of the inferior turbinate). Bronchoscopy revealed approximately 80% stenosis of the subglottis (grade III, Cotton-Myer grading system) (FIGURE 2 arrows) and long-segmental subglottic granulomatous masses involving the glottis (stage IV, McCaffrey classification system) (FIGURE 3 arrow). An impending life-threatening subglottic stenosis was noted. Therefore, the patient underwent ventilation bronchoscopy and laryngotracheal reconstruction