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
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