Acquired subglottic stenosis is mostly secondary to trauma, and can also arise in the context of chronic inflammatory diseases1. Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by small vessel vasculitis and granulomatous lesions of the respiratory tract. Approximately 25% of patients with GPA develop subglottic stenosis2.
CASE PRESENTATION:A 48-years-old female, former-smoker (6 pack-years), with chronic rhinosinusitis and without surgical history. She was referenced due to episodes of shortness of breath and stridor, with 2 months of evolution. On physical examination, she presented dysphonia and saddle nose. Rhinoscopy showed nasal septum destruction and mucosa inflammation with lots of crusts. Cervical-thoracic CT scan revealed extensive sinusopathy and concentric subglottic thickening, in a 35mm longitudinal extension and 5mm thickness, just below the cricoid cartilage resulting in reduced airway caliber. Flexible bronchoscopy showed multiple membranes covering the mucosa with inflammatory and exudative aspects, resulting in about 50% of reduction in the subglottic tracheal lumen. The patient underwent rigid bronchoscopy (RB); bronchial biopsies and mechanical dilation were performed. Bronchial biopsies were compatible with a nonspecific inflammatory process. Repeated antineutrophil cytoplasmic antibody (ANCA) tests were negative. Three months later, she experienced clinical worsening with emission of circumferential membranous rings (molds of tracheal mucosa); bronchoscopy was repeated with a 20% stenosis, immediately distal to the vocal cords.Limited GPA was diagnosed and intravenous methylprednisolone pulses were done; subsequently she maintained immunosuppressive therapy with methotrexate and prednisolone. The patient evolved with subglottic stenosis recurrence and a new RB was needed, with dilation and mitomycin-C instillation. Given the failure of immunosuppressive therapy, she was proposed for rituximab.DISCUSSION: This case illustrates that subglottic stenosis could be considered a localized form of GPA, usually ANCA-negative, characterized by refractoriness to conventional therapy and a relapsing course3.CONCLUSIONS: Subglottic stenosis due to GPA is associated with high morbidity. Endoscopic therapies provide symptomatic relief, however recurrence is frequent. The therapeutic approach should be customized on a case-by-case basis.
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