Management of airway manifestations of relapsing polychondritis: case reports and review of literature

Abstract

Study objective: To report the first series of patients with severe airway manifestations of relapsing polychondritis (RP) that were managed successfully with self-expandable metallic stents, and to review the literature.

Design: Retrospective review of medical records, and current clinical follow-up.

Setting: Tertiary care referral hospital.

Patients: All patients with airway manifestations of RP that were managed with self-expandable metallic stents at our institution.

Results: All five patients (four women and one man; age, 40 to 69 years old) had severe airway manifestations, and three of them required mechanical ventilation. Spirometry with flow-volume curves showed severe combined obstructive and restrictive ventilatory defects. Bronchoscopy revealed dynamic collapse of the proximal airways. Diagnosis was made 8 months to 13 years after the first symptom of the disease. Pharmacotherapy included prednisone, methotrexate, cyclosporine, and dapsone. A total of 17 self-expandable metallic stents of varying sizes were placed using flexible bronchoscope from 4 to 19 years after the first symptom. The overall outcome was favorable in four patients. Three patients have survived without ventilatory support 16 to 18 months following the first stent placement, and the fourth patient survived for 20 months without ventilatory support before she died. The fifth patient, who was receiving mechanical ventilation, died in 1 week probably due to persistent dynamic collapse of the airways distal to the stents.

Conclusion: Self-expandable metallic tracheobronchial stents should be considered in the management of airway manifestations of RP, especially in patients who require mechanical ventilation.