Abstract Introduction Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis, is a necrotizing vasculitis of small- to medium-sized vessels typically presenting with pulmonary-renal and upper airway involvement. Subglottic stenosis (SGS) is a recognized but uncommon isolated manifestation without pulmonary or renal disease. We report a pediatric case of GPA presenting solely with SGS. Case A 13-year-old male with mild intermittent asthma and seasonal allergies presented to the Pediatric Pulmonary Clinic with several months of exertional dyspnea, wheezing, and intermittent noisy breathing. He had no history of intubation or prior severe respiratory illness. Physical examination revealed intermittent wheeze in the right upper lung field with otherwise normal aeration. Pulmonary function testing revealed a fixed large airway obstruction, with a normal diffusing capacity and normal plethysmography results. The chest radiograph was unremarkable, but the soft tissue neck X-ray showed mild subglottic irregularity. Otolaryngology and Pulmonary airway evaluation with combined flexible and rigid bronchoscopy revealed Grade III subglottic stenosis (∼80%), improved to Grade I after dilation. Bronchoalveolar lavage (BAL) culture results were normal; pathology revealed fibrous tissue with both acute and chronic inflammation. Despite initial improvement, the patient developed recurrent symptoms requiring repeat balloon dilations, cold knife techniques and subglottic steroid injection. Due to the association of SGS with GPA, antineutrophil cytoplasmic antibodies (ANCA) testing was performed, which revealed an elevated proteinase 3 (PR3) antibody level (245 U/mL) with normal myeloperoxidase (MPO) levels. Further evaluation demonstrated chronic sinus disease on maxillofacial computed tomography but normal chest and abdominal imaging, and no renal involvement. He met diagnostic criteria for GPA with isolated SGS. He was started on Rituximab and Prednisone, with Bactrim prophylaxis. He continues to require serial endoscopic management for restenosis, however, with decreasing frequency since initiating immunomodulation. Discussion GPA is rare in children, comprising only 3-7% of all cases, and typically shows a female predominance. While systemic involvement of the respiratory and renal tracts is classic, isolated SGS may precede or represent the only manifestation of disease. Recognition of fixed large airway obstruction on spirometry should prompt an airway evaluation, particularly in patients with unexplained dyspnea and normal chest imaging results. Early identification of GPA-associated SGS enables the timely initiation of immunosuppressive therapy to prevent disease progression and morbidity. Pediatric patients presenting with idiopathic subglottic stenosis should raise suspicion for underlying GPA, even in the absence of pulmonary or renal findings. This abstract is funded by: None
Pant et al. (Fri,) studied this question.