Relapsing polychondritis (RP) is a rare disease of unknown etiology causing systematic inflammation and resulting in destruction of cartilaginous tissues. We describe here an 18-year-old Japanese woman who developed severe airway stenosis as the initial symptom with auricular, nasal, and ocular inflammation. The effect of high dose oral steroid, methylprednisolone pulse therapy, and cyclophosphamide was temporary and her conditioning was worsening. Finally we added methotrexate to the immunosuppressive treatment and achieved reduction of disease activity. This case illustrates the potentially fatal sudden onset of airway inflammation that can occur with this disorder, and the effectiveness of methotrexate.
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