Management of patients with relapsing polychondritis and airway complications

Objectives: We encountered 7 patients with relapsing polychondritis complicating tracheobronchial narrowing. To describe the method of management and clinical outcome, we retrospectively reviewed medical records. Methods: Relapsing polychondritis was diagnosed according to the criteria of McAdam modified by Daminani and Levine. Airway complication was documented by 3-dimensional computed tomography scans and bronchoscopy. Clinical characteristics, diagnostic test results, records of consultation with the rheumatology department, treatment modalities, and clinical outcomes were investigated. Results: Among the 7 patients (4 women and 3 men; 18-69 years old), mechanical ventilation was required in 5. Patients were treated with high-dose corticosteroids for acute exacerbation and maintained with low-dose prednisolone with methotrexate or cyclophosphamide during follow up. Currently, 6 patients have survived without ventilatory support and 3 patients without tracheostomy. Four patients underwent endobronchial stenting, and the number of interventions was 1 to 6 per patient. During follow up (1.5-53 months), clinical outcome was favorable in 6 patients, but 1 patient died of pneumonia and respiratory failure. Conclusion: High doses of corticosteroids on acute exacerbation followed by maintenance low-dose prednisolone with immunosuppressants ± endobronchial stenting could be considered a therapeutic option in patients with relapsing polychondritis complicating tracheobronchial stenosis.