B35-32 Relapsing Polychondritis With Tracheal Stenosis

Abstract Introduction Relapsing polychondritis (RP) is a rare, immune-mediated disorder characterised by recurrent inflammation of cartilaginous and proteoglycan-rich tissues. Clinical manifestations are heterogeneous; typical features include auricular and nasal chondritis, seronegative polyarthritis, ocular inflammation and can also include inflammation of the cartilaginous tissues of the larynx, trachea and bronchi. It may progress to long-lasting atrophy and/or deformity, with tracheobronchial disease occurring in 20-50% of cases. The diagnosis of RP is based on a set of clinical evidence and imaging studies, and the treatment remains empirical. Existing data supports the use of NSAIDs and low dose corticosteroids in mild cases and high dose corticosteroids and conventional immunosuppressants (e.g. Methotrexate) or biologic therapies (e.g. TNF-α inhibitor) in severe cases. Case A 31-year-old African man presented with loud stridor on a background of a 1 year history of progressive cough and exertional dyspnoea, as well as an episode of uveitis 3 months prior. He had multiple emergency department presentations with repeated courses of oral corticosteroids and antibiotics for presumptive asthma without sustained benefit. On presentation, nebulised adrenaline and systemic steroids did not improve his symptoms. Inflammatory markers were elevated (ESR 40 mm/h, CRP 30 mg/L). Urgent computed tomography of his thorax and neck demonstrated focal sub-glottic tracheal narrowing with extensive anterior tracheal thickening and calcified nodularity, with marked narrowing of both main bronchi. Pulmonary function testing showed a severe, non-reversible obstructive defect with preserved TLCO. Differential diagnoses included RP, sarcoidosis, and granulomatosis with polyangitis. Rigid bronchoscopy revealed subglottic stenosis 3 cm below the vocal cords and diffuse tracheal nodularity with a cobblestone appearance. Histology and cytology were negative for infection and malignancy. The patient remained intubated post-procedure as the airway was felt to be unstable. The diagnosis of RP was established following multidisciplinary evaluation between rheumatology, respiratory, cardiothoracic and anaesthetic teams. He had a prolonged ICU admission which was complicated by a hospital-acquired pneumonia, complex seizures, acute CMV infection and critical care myopathy. After 2 months of immunosuppressive treatment, a repeat bronchoscopy showed significant improvement in the subglottic stenosis, albeit demonstrating a “thickened” trachea. Prednisolone was tapered over four months to a maintenance dose of 7.5 mg daily alongside ongoing two-weekly Infliximab with good improvement. Conclusion This case highlights tracheobronchial RP as a medical and surgical emergency requiring early recognition, rapid airway assessment, and coordinated multidisciplinary care. Corticosteroids, biologic therapy and timely airway intervention (including bronchoscopy and tracheostomy) may form key components of management. This abstract is funded by: NA