Two Cases of the Relapsing Polychondritis with Hoarseness and Dyspnea

概要

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Relapsing polychondritis (RP) is a systemic disorder of unknown etiology characterized by inflammation of the cartilaginous structure. We had two cases of RP. The first patient (50 year-old woman) and the second patient (33 year-old woman) were admitted with hoarseness and dyspnea because of subsequently developing glottic and tracheal stenosis. These cases were diagnosed by criteria of RP or physical symptoms, biopsy and effects of steroid therapy. We analyzed clinical features, laboratory data, pathology, therapy and prognosis in 56 cases in the literature in Japan. The cases that were diagnosed and received steroid therapy within a year had good prognosis. It is necessary to follow up for at least a year, when steroidos are decreased and abandoned. It is also important that we do appropriate respiratory management because the upper airway stenosis can be fatal.