Abstract:

Objective  To investigate the clinical characteristics of relapsing polychondritis(RP) with the involvement of respiratory tract. Methods  The clinical features, tests and treatment of RP with airway involvement in 3 patients were retrospectively reviewed. Results  Three RP cases of typical symptoms were confirmed by biopsy. CT scan with 3D reconstruction and bronchoscopy revealed the range and severity of the lesion. After initial medications, such as corticosteroids and immunosuppressive drugs, the symptoms in all cases alleviated significantly for a while but tended to recur and deteriorate later. During the follow-up, one recurrent case adopted laryngotracheal reconstruction in which pedicled surgical flaps was applied and a long-term stable condition persisted. Meanwhile, 2 cases died in the second and fifth year respectively after the diagnosis. Conclusion  RP with airway involvement is a rare disease affecting multiple systems in the body. The diagnosis is made on the clinical features, imaging studies and pathological examination. Early treatment with corticosteroids and immunosuppressive agents might achieve a relatively satisfying result at the beginning. However, in the later stage, invasive approaches, including tracheotomy, laryngotracheal reconstruction, should be applied for a better prognosis.

Key words: Relapsing polyehondritis; Trachdal stenosis; Early diagnosis