Abstract Introduction Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by granulomatous inflammation and necrotizing vasculitis affecting multiple organs, particularly the respiratory tract. Tracheobronchial stenosis (TBS) is a severe and potentially fatal complication that may occur as an initial or isolated manifestation, even without systemic involvement. The diagnostic gold standard is direct visualization through bronchoscopy. Treatment typically combines immunosuppressive therapy to control disease activity with interventional procedures to relieve airway obstruction. Currently, no standardized clinical practice guidelines exist for the management of GPA-associated TBS. Methods We conducted a retrospective study at the National Institute of Respiratory Diseases (INER) analyzing patients diagnosed with GPA and TBS between 2012 and 2023. Adults (≥18 years) with confirmed diagnoses were included. Data collected included stenosis characteristics, systemic manifestations, bronchoscopy findings, and treatment outcomes. The objective was to describe clinical evolution and evaluate the impact of interventions on stenosis recurrence. Results Data from 41 patients were analyzed; 70.7% were female, with a mean age of 48 years and a mean age at GPA diagnosis of 41 years. Localized vasculitis was the predominant form (56%). At diagnosis, 47% were C-ANCA positive. Stenosis was initially identified by bronchoscopy in 58.8% of cases, while 58% had stenosis prior to GPA diagnosis. Among patients without initial stenosis, the mean time from GPA diagnosis to stenosis was 300 days. Subglottic stenosis was the most common site, followed by bronchial involvement; 10 of 31 patients had multilevel disease. A total of 266 bronchoscopies were performed—57% in symptomatic patients—with a complication rate of 8%. Across all procedures, 454 dilatations were carried out, evenly divided between balloon and mechanical techniques. The highest number of dilatations in a single patient was 44. Among 201 bronchoscopies, 95 biopsies were performed (47%), revealing active vasculitis in only three cases, all with concurrent systemic activity. Regarding treatment, methotrexate was associated with the highest number of relapses (89), followed by rituximab (48). Conclusions This study provides a detailed characterization of GPA-associated TBS in our center. Early recognition and multidisciplinary management are essential to improve outcomes. Standardization of diagnostic criteria, stenosis evaluation, and treatment protocols across specialties is urgently needed to reduce recurrence and minimize complications. This abstract is funded by: None
Salazar et al. (Fri,) studied this question.