A35-05 Granulomatosis With Polyangitis Presenting as Subglottic Stenosis

Abstract Granulomatosis with polyangiitis (GPA) is a rare multi-system disease characterized by non-caseating granulomatous vasculitis. It can involve any organ system; however, it commonly affects the lungs and kidneys. Over the course of the disease, pulmonary involvement occurs in approximately 90% of patients while only 9% have sole pulmonary involvement. Fever and weight loss are the most common presenting symptoms, and pulmonary manifestations include cough, dyspnea, chest pain, and, rarely, subglottic stenosis (SGS). We report a case of female patient who initially experienced sinonasal symptoms followed by cough, wheezing, and stridor. She was diagnosed with granulomatosis with polyangiitis after a laryngoscopy revealed subglottic stenosis and autoimmune serology was positive for MPO-ANCA. A 26-year-old Hispanic female with a history of severe hypertension since adolescence, an atrophic left kidney, and chronic nasal congestion presented with progressive upper and lower airway disease. She first experienced sinonasal symptoms, including obstruction, crusting, and septal deviation, that ultimately required septoplasty and turbinectomy in 2020. By age 25, she developed persistent cough, wheezing, and stridor unresponsive to bronchodilators or corticosteroids. Pulmonary function testing suggested obstructive physiology, and laryngoscopy revealed subglottic stenosis. Cross-sectional imaging demonstrated airway narrowing with bronchial wall thickening and a thin web within the left mainstem bronchus, while bronchoscopy confirmed scarring at the carina, circumferential narrowing of the left mainstem bronchus, and multifocal airway stenoses. Biopsies showed chronic inflammation without granulomas or necrosis. Autoimmune serologies revealed a strongly positive MPO-ANCA with negative PR3-ANCA. Taken together, her chronic sinonasal disease, subglottic stenosis, multifocal bronchial involvement, and positive MPO-ANCA were diagnostic of granulomatosis with polyangiitis with pulmonary involvement, limited form, despite nondiagnostic histopathology. She was started on azathioprine, and balloon dilation of her subglottic stenosis was deferred pending immunosuppressive control. This case highlights a limited form of GPA with SGS and possible treatment modalities. While over 90% of patients with GPA present with upper and/or lower respiratory tract symptoms, 20% develop SGS, which reflects the chronic phase of airway obstruction from GPA. The acute phase occurs from fixation of the vocal folds from inflammation of the laryngeal joints. Patients with symptoms such as stridor, hoarseness, and dyspnea necessitate urgent evaluation for impending airway obstruction. Treatment for GPA typically consists of induction therapy, including glucocorticoids, followed by a maintenance regimen with the use of immunosuppressive medications. Patients with symptomatic SGS are usually treated with bronchoscopic techniques, including dilation, laser debridement, and corticosteroid injection. Refractory disease may require tracheostomy. This abstract is funded by: None