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抄録

強皮症は皮膚および内臓諸臓器の線維化と末梢循環障害を主徴とする結合織疾患である。原因は依然不明で、既存のステロイドや免疫抑制剤に抵抗性であり、皮膚線維化だけでなく臓器病変も含めた治療法の確立は急務である。
一方、マスト細胞は寄生虫に対する防御機構やアレルギー性疾患などとの関与は良く知られているが、最近になり自然免疫から獲得免疫の移行に重要である事や関節リウマチの病態にも関与し治療標的の可能性が示唆される様になっている。本稿では、マスト細胞の強皮症病態への関与について我々の知見も踏まえて概説し、マスト細胞を標的とした強皮症治療の可能性について述べる。

Summary

Systemic sclerosis is characterized by tissue fibrosis, obliterative microangiopathy and immune abnormalities. The etiology of SSc is largely unknown and is known to be resistant to existing corticosteroid and immunosuppressive drugs. Therefore, establishment of a treatment strategy especially for SSc patients with organ involvement is strongly desired. Mast cells are widely recognized as effector cells in allergic disorders and other IgE–mediated immune responses. However, recently, mast cells have become known to play a role in bridging innate immunity and adaptive immunity. Additionally, there is growing evidence of mast cell to be involved in pathogenesis of rheumatoid arthritis, and is expected as a novel therapeutic target. We describe here the role of mast cell in SSc pathology and suggest as a novel therapeutic target.