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- W2336194645 abstract "抗MDA5抗体は皮膚筋炎特異的抗体であり，同抗体陽性例は筋症状が少なく，高率に急速進行性間質性肺炎を併発し予後不良であること，高フェリチン血症・肝胆道系酵素上昇を伴うなど特徴的な臨床像を呈する．抗MDA5抗体陽性患者と陰性皮膚筋炎患者の治療前血清サイトカインを比較したところ前者では有意にIL-6, IL-18, M-CSF, IL-10が高値を示し，IL-12, IL-22は低値を示した．これらのことから，抗MDA5抗体陽性例においては単球・マクロファージの異常活性化が病態の背景に存在すると考えている．抗MDA5抗体陽性例は一旦酸素投与が必要な呼吸不全状態になると極めて救命率が低いため，診断後可及的速やかに治療介入が必要であると考えられる．ステロイド大量療法・シクロスポリン内服・シクロホスファミド間歇静注療法（IVCY）の3剤を併用した強力免疫抑制レジメンを用いることで，同抗体陽性例の予後が改善した．特に疾患活動性を反映することが報告されている血清フェリチン値は，IVCY投与約2週後に低下する傾向を認め，IVCYが同抗体陽性例の治療においてキードラッグとなることが示唆された．" @default.
- W2336194645 created "2016-06-24" @default.
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- W2336194645 date "2013-01-01" @default.
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- W2336194645 title "Anti-MDA5 (melanoma differentiation-associated gene 5) antibody and dermatomyositis with rapidly progressive interstitial pneumonia" @default.
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- W2336194645 doi "https://doi.org/10.2177/jsci.36.71" @default.
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