Abstract Introduction Airway involvement in granulomatosis with polyangiitis (GPA) occurs in up to 20–25% of patients but typically does not dominate disease trajectory. Subglottic stenosis (SGS) usually develops later and is often responsive to immunosuppression and endoscopic therapy. However, a subset—especially younger patients—experience aggressive, recurrent SGS that remains refractory. We present a case of early-onset, rapidly recurring SGS requiring multiple airway interventions despite intensive immunosuppression. Case A 22-year-old male with PR3-ANCA–positive GPA initially developed recurrent epistaxis and nasal crusting at age 17. After outpatient treatment for presumed pneumonia, he presented with hemoptysis, malaise, and tea-colored urine, requiring PICU admission for respiratory distress and acute kidney injury. Workup revealed PR3-ANCA 217 U with normal complement levels. Chest CT demonstrated bilateral nodules and opacities consistent with diffuse alveolar hemorrhage, and renal biopsy confirmed necrotizing crescentic glomerulonephritis.He received high-dose IV methylprednisolone, rituximab, and six months of cyclophosphamide. Transition to mycophenolate for maintenance was stopped following a severe airway flare with 70–80% subglottic narrowing requiring urgent dilation. He underwent re-induction with cyclophosphamide, then maintenance rituximab, later changed to azathioprine.Despite stable renal and pulmonary status, he developed progressive SGS requiring 7–8 endoscopic dilations over 3–4 years, with shortening intervals. Recent bronchoscopy showed circumferential SGS extending to the second tracheal ring with active inflammation, consistent with refractory localized disease. Discussion SGS is a significant complication of GPA, particularly in those diagnosed at younger ages (1,2). Importantly, airway disease may progress independently of systemic disease markers, reflecting localized vasculitic injury and fibrostenosis. Standard immunosuppression—including corticosteroids, cyclophosphamide, rituximab, and antimetabolites—often fails to prevent restenosis (3–5). Endoscopic dilation provides short-term airway relief but does not modify underlying pathophysiology and may contribute to scarring. In selected patients, laryngotracheal resection offers improved durability, though some continue to require adjunctive dilations (2,3).This case underscores the need for early airway-focused surveillance, close coordination between rheumatology and otolaryngology, and timely evaluation for definitive airway reconstruction once systemic inflammation is controlled. References: 1. Quinn KA et al. Rheumatology (Oxford). 2019;58(12):2203–2211. 2. Gluth MB et al. Laryngoscope. 2003;113(8):1304–1307. 3. Blackabey V et al. AME Case Rep. 2018;2:17. 4. Ugan Y et al. Eur J Rheumatol. 2018;5(1):69–71. 5. An J-W et al. Medicina (Kaunas). 2021;57(5):423. This abstract is funded by: none
Mustafa et al. (Fri,) studied this question.