[Relapsing polychondritis with predominant respiratory involvement treated with Dapsone :value of pulmonary function...

A 31-year-old patient had developed lesions in the nasal cartilage during infancy, followed by laryngotracheal lesions when 9 years old. The latter lesions recurred when he was 21, followed several years later by an affection of the main left bronchus and the diagnosis of relapsing polychondritis. Initial respiratory function tests showed mainly a reduction in maximum inspiratory flow, due to sequelae in the upper respiratory tract. Repeated tests were able to demonstrate lesions in the main left bronchus, determining severe expiratory slowing. No significant improvement occurred after one year of corticoïd therapy. Treatment with sulfones (Disulone) produced clinical improvement (reappearance of a vesicular murmur in the left lung) and a twofold increase in FEV1. Functional improvement was even better demonstrated by successive flow-volume curves.