Bertuccio Francesco Rocco, Valente Davide, Baio Nicola, Tomaselli Stefano, Saracino Laura, Sciandrone Gaetano, Milanesi Alessandra, Delvino Paolo, Codullo Veronica, Corsico Angelo Guido, Stella Giulia Maria
Unit of Respiratory Disease, Cardiothoracic and Vascular Department, IRCCS Policlinico San Matteo, Viale Golgi 19, 27100 Pavia, Italy.
Department of Internal Medicine and Pharmacology, University of Pavia, 27100 Pavia, Italy.
J Clin Med. 2025 Feb 25;14(5):1532. doi: 10.3390/jcm14051532.
Tracheobronchial stenosis is a significant complication in granulomatosis with polyangiitis (GPA), a systemic vasculitis that primarily affects the upper respiratory tract, kidneys, and lungs. The involvement of the tracheobronchial tree in GPA leads to airway narrowing, which can result in severe respiratory symptoms and increased morbidity, often requiring prompt diagnosis and management to prevent life-threatening airway obstruction. We present the case of a 28-year-old male with mild exertional dyspnea, stridor, and retropharyngeal sputum. Clinical investigations revealed subglottic and bronchial concentric stenosis with granulomatous inflammation. A diagnosis of granulomatosis with polyangiitis (GPA) with isolated tracheobronchial stenosis (TBS) was confirmed. Given the severity of airway obstruction, multidisciplinary management was initiated, combining rigid bronchoscopy with systemic immunosuppressive therapy. Post-intervention follow-up demonstrated significant airway improvement and maintained remission after two years. This case highlights TBS as a potentially debilitating GPA manifestation requiring a combination of systemic and endoscopic therapies. Further studies are needed to optimize therapeutic approaches and improve outcomes in GPA-associated TBS.
气管支气管狭窄是肉芽肿性多血管炎（GPA）的一种重要并发症，GPA是一种主要累及上呼吸道、肾脏和肺部的系统性血管炎。气管支气管树受累于GPA会导致气道狭窄，进而引发严重的呼吸道症状并增加发病率，常常需要及时诊断和处理以防止危及生命的气道梗阻。我们报告一例28岁男性病例，其有轻度劳力性呼吸困难、喘鸣和咽后痰液。临床检查发现声门下和支气管同心性狭窄伴肉芽肿性炎症。确诊为肉芽肿性多血管炎（GPA）伴孤立性气管支气管狭窄（TBS）。鉴于气道梗阻的严重性，启动了多学科管理，将硬质支气管镜检查与全身免疫抑制治疗相结合。干预后的随访显示气道有显著改善，且两年后维持缓解状态。该病例凸显了TBS是一种可能导致身体衰弱的GPA表现形式，需要全身治疗和内镜治疗相结合。需要进一步研究以优化治疗方法并改善GPA相关TBS的治疗效果。
