Objective: To investigate the clinical cause, treatments and prognosis of laryngotracheobronchial involvement in relapsing polychondritis (RP).

Methods: Thirteen patients of RP with laryngotracheobronchial involvement were review retrospectively.

Results: From 1983 to 1998, 20 patients with RP were managed in this hospital. Of them, 13 (65%) involving the laryngotracheobronchial region were observed. Respiratory tract lesions might be diffuse or localized and occurred in larynx (46%), trachea (69%) and bronchus (69%). Corticosteroids, immunosuppressive agents and dapsone were used in these patients. Tracheotomy was performed in 4 patients with airway obstruction. Montgomery T-tube or self-expanding metal stent were used in 2 patients with tracheal collapse. Of 13 case, 2 died from respiratory complications. The 5-years survival rate was 78.6%.

Conclusions: Respiratory tract involvement is the most severe manifestation of the relapsing polychondritis and the main cause of death despite aggressive medical therapy or tracheotomy.