Abstract Introduction Granulomatosis with polyangiitis (GPA) is a multisystem small- and medium-vessel vasculitis most often affecting the respiratory tract and kidneys. Endobronchial involvement, including tracheobronchial stenosis, occurs in a minority of patients and often leads to symptomatic dyspnea (1). While cases of complete bronchial stenosis are rare, they typically result in compromised aeration. Preserved ventilation of a completely obstructed lobe has not been described. We report a novel case of complete left upper lobe (LUL) bronchial stenosis with maintained ventilation in GPA. Description A 69-year-old man with GPA initially presented with cavitary lung lesions and multifocal nodules. Eight years after the initial diagnosis, he developed recurrent symptoms with new ground-glass opacities. Bronchoscopy demonstrated inflammatory changes with complete obstruction of the LUL takeoff. Imaging and physiologic assessment showed that the LUL remained well-ventilated despite complete anatomic stenosis. Serial bronchoscopies revealed progressive “vanishing airway” changes. Multiple interventions—including debridement, balloon dilation, and repeated stent placements—were complicated by difficult anatomy, mucous plugging, granulation, and stent fracture. Ultimately, a covered Bona stent achieved improved patency of the LUL, though partial obstruction persisted. Discussion Tracheobronchial stenosis is an uncommon but serious manifestation of GPA. In this case, the presence of a well-ventilated left upper lobe despite complete obstruction of the lobar takeoff represents a rare and physiologically intriguing phenomenon. Preservation of ventilation despite complete lobar obstruction likely reflects collateral pathways such as pores of Kohn or canals of Lambert. This case underscores that obstruction does not always equate to functional loss, and early recognition of airway involvement is essential to prevent irreversible remodeling. Timely referral to interventional pulmonology enables therapeutic options such as stenting to restore airway patency (2)(3). References: 1) Quinn KA, Gelbard A, Sibley C, et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology (Oxford). 2019;58(12):2203-2211. doi:10.1093/rheumatology/kez217. 2) Terrier B, Dechartres A, Girard C, et al. Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis—results from a multicentre experience. Rheumatology (Oxford). 2015;54(10):1852-1857. doi:10.1093/rheumatology/kev129. 3) Martinez Del Pero M, Jayne D, Chaudhry A, Sivasothy P, Jani P. Long-term outcome of airway stenosis in granulomatosis with polyangiitis (Wegener granulomatosis): an observational study. Rheumatology (Oxford). 2015;54(10):1852-1857. This abstract is funded by: None
Abdalla et al. (Fri,) studied this question.