Tracheobronchial involvement as a sole manifestation of relapsing polychondritis

Study Objectives: To report the clinical, imaging, and pathologic manifestations of case series of patients in whom the only systemic expression of relapsing polychondritis (RP) was their airway complications. Design: Retrospective review of the medical records of all patients with respiratory complications of RP between 1995 and 2007. Setting: Tertiary care, university-affiliated hospital. Results: Three patients with RP had just lower airway manifestations as the only sign of their RP. All 3 were women, aged 44, 49, and 54 years. All had an abnormal chest computed tomography scan, although 2 had a completely normal chest x-ray. All had positive tracheal biopsy, which was consistent with the diagnosis of respiratory chondritis. Pulmonary function tests showed severe reduction in forced expiratory volume in 1 second in all patients. Bronchoscopy revealed tracheal narrowing with variable degrees of inflammation and collapsibility in all patients. Two of the 3 patients underwent tracheal and bronchial stent insertion. Pharmacotherapy included prednisone, methotrexate, cyclophosphamide, and leflunomide. The overall outcome was poor. Two patients died as a result of respiratory complications, 25 and 30 months from diagnoses, and 1 is still alive with follow-up of 85 months after presentation. Conclusions: Lower airway manifestations of RP can be the only sign of the disease. RP has to be considered in the differential diagnosis of patients with recent onset of progressive dyspnea and severe airflow limitation even without other systemic signs of cartilage damage. © 2010 by Lippincott Williams & Wilkins.