P120 A difficult-to-treat case of granulomatosis with polyangiitis with large airway involvement

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Abstract Introduction Granulomatosis with polyangiitis (GPA), a subtype of ANCA-associated vasculitis, typically affects the upper respiratory tract, lungs, and kidneys. While pulmonary parenchymal involvement is well known, tracheobronchial manifestations are under-recognised and often overlooked due to their varied clinical presentations. This case report explores a challenging GPA presentation with progressive large airway disease — a complication that remains diagnostically elusive and therapeutically resistant. The significance lies in highlighting the diagnostic complexity, therapeutic limitations, and the need for heightened awareness and multidisciplinary management when large airways are involved. Case description A 51-year-old male was diagnosed with GPA in August 2023 following a clinical presentation of haemoptysis and a preceding history of nasal congestion, epistaxis, weight loss, and a markedly raised PR3 level of 49 (normal <4). Initial investigations revealed multifocal lung consolidations, bronchial mural thickening, and a potential right middle lobe collapse. Treatment began with systemic corticosteroids and intravenous cyclophosphamide. Following induction, he was transitioned to mycophenolate mofetil and tapering steroids. However, his condition worsened with increasing cough, dyspnoea, and audible wheeze. Bronchoscopy showed ulceration and granulation within the bronchi. Repeat imaging with CT chest and PET-CT revealed persistent pulmonary infiltrates and worsening collapse of the right middle lobe. His immunosuppressive regimen was intensified with additional cyclophosphamide and rituximab infusions, yet symptoms persisted. A subsequent CT scan revealed complete occlusion of the left upper lobe bronchus and segmental collapse. He required multiple admissions for respiratory support and infection management, including treatment for Pseudomonas. Avacopan was introduced to minimise corticosteroid burden. Though disease progression slowed, symptoms remained refractory. Bronchoscopic airway dilatation resulted in partial symptomatic improvement. He continues on maintenance rituximab, avacopan and corticosteroids. Discussion This case posed significant clinical challenges due to the progressive nature of GPA involving large airways. The decision to escalate immunosuppressive therapy — including cyclophosphamide, rituximab, and avacopan — was guided by both radiological findings and symptomatic deterioration. Despite aggressive medical management, airway compromise worsened, emphasising the refractory nature of tracheobronchial GPA. Bronchoscopic evaluation and biopsies were crucial in diagnosing large airway involvement, a commonly missed phenotype of GPA. Multidisciplinary input from respiratory medicine, rheumatology, and interventional pulmonology facilitated a tailored approach, including airway dilatation, which offered transient relief. Learning from this case underscores the limitations of conventional GPA treatment in managing airway disease. Immunosuppressive escalation may not always halt progression, and adjunctive bronchoscopic or surgical interventions become necessary. Questions remain around optimal treatment algorithms, timing of interventions, and long-term airway monitoring. This case should prompt deeper discussion on diagnostic strategies — including earlier use of bronchoscopy — and treatment personalisation. It also highlights the need for clearer recognition of airway GPA as a distinct clinical challenge within ANCA vasculitis care pathways. Whether alternative immunomodulatory agents or targeted airway therapies might enhance outcomes is an area ripe for exploration. Key learning points Recognition of airway GPA: Tracheobronchial involvement in GPA can be underdiagnosed due to its subtle and varied presentation. Clinicians should consider it in persistent respiratory symptoms despite standard immunosuppressive therapy. Diagnostic value of bronchoscopy: Bronchoscopy with biopsy proved pivotal for diagnosis. In cases with unexplained respiratory decline, early endoscopic evaluation is essential to detect airway ulceration, granulation, or stenosis. Limits of immunosuppression: Despite maximal therapy, disease progression continued. This highlights the need to accept immunosuppressive ceiling effects and consider interventional modalities early. Multidisciplinary coordination: The patient’s care required close collaboration across specialties, showcasing the importance of integrated management in complex vasculitis cases. Learning objectives 1. Understand the varied presentation and progression of large airway GPA. 2. Explore diagnostic pathways including bronchoscopy and advanced imaging. 3. Evaluate the therapeutic limitations of immunosuppression in airway involvement. 4. Discuss timing and integration of interventional techniques. 5. Encourage development of targeted treatment strategies for airway-dominant GPA.

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