[Tracheal stenosis as a initial manifestation of relapsing polychondritis. Case report]

Relapsing polychondritis is a rare multisystemic disease, of unknown origin, characterized by recurrent swelling, destruction of cartilages and connective tissue mainly of the ear and nose cartilages. Complications of the airways (laryngeal and tracheal stenosis) occur late in the disease. We present a case of a male with relapsing polychondritis which appeared initially as a tracheal stenosis (laryngeal stridor, exertional dyspnea) followed by cutaneous lesions, swelling of the ear and nose cartilage (deformity of nose septum in the shape of a saddle). Computerized tomography of the neck showed a decrease in diameter of the trachea about 50%, bronchoscopy revealed a concentric diminution of the tracheal lumen about 50% that involved the right and left main bronchi. A biopsy of the ear cartilage showed signs of chondritis. The patient responded satisfactorily to steroids and dilatation of stenosis; however, one year later he died of respiratory insufficiency. Tracheal stenosis is a sign of ominous prognosis in relapsing polychondritis.