Subglottic Stenosis in Wegener's Granulomatosis Limited to the Head and Neck Region

Abstract

Subglottic stenosis as a complication of Wegener's granulomatosis (WG) is a relatively rare lesion and is difficult to treat surgically once stenosis becomes sufficiently severe to cause inspiratory dyspnea. Thus, it is important to diagnose WG in its early stages to prevent troublesome subglottic stenosis from developing by initiating immunosuppressive therapy. The authors report on a 30-year-old woman suffering from subglottic stenosis of sudden onset due to protracted WG limited to the head and neck region. She had had exudative otitis media for 13 years and saddle nose and nasal crusting for five years. Repeated biopsies of the nasal mucosa and enzyme-linked immunosorbent assays for cytoplasmic patterns of antineutrophil cytoplasmic autoantibody (cANCA) had failed to establish the diagnosis. However, further histologic examination of the nasal mucosa showed vasculitis, and indirect immunofluorescence delected the presence of cANCA. Thus, the diagnosis of WG was confirmed 13 years after the appearance of the initial symptoms in the ear. The patient was given prednisolone (60 mg/day for five days), which greatly relieved the subglottic stenosis. The prednisolone dosage was then tapered to 5 mg/day.