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おわりに

強皮症は皮膚線維化だけでなく、多彩な臓器病変を来たし得る難治性結合織病であるが、自然軽快を含めた皮膚硬化の進行の程度や合併する臓器病変は多様性に富むため、その治療法の確立が困難な一因となっている。しかし今回の我々の結果は、マスト細胞による強皮症の皮膚線維化や肺高血圧病態への強い関与を示唆すると共に、治療標的とすることで新規治療法開拓の可能性をも提示するものである。

文献

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