Yamaoka Kunihiro, Saito Kazuyoshi, Hanami Kentaro, Nakayamada Shingo, Nawata Masao, Iwata Shigeru, Azuma Taeko, Tanaka Yoshiya
First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Yahata-nishi-ku, Kitakyushu 807-8555, Japan.
Mod Rheumatol. 2007;17(2):144-7. doi: 10.1007/s10165-006-0548-5. Epub 2007 Apr 20.
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.
复发性多软骨炎（RP）是一种病因不明的罕见疾病，可引起系统性炎症并导致软骨组织破坏。我们在此描述一名18岁的日本女性，她以严重气道狭窄为首发症状，并伴有耳、鼻和眼部炎症。高剂量口服类固醇、甲泼尼龙冲击疗法和环磷酰胺的效果是暂时的，她的病情不断恶化。最后，我们在免疫抑制治疗中加入了甲氨蝶呤，疾病活动度得以降低。该病例说明了这种疾病可能会突然发生致命的气道炎症，以及甲氨蝶呤的有效性。
