2018 Fiscal Year Final Research Report
Autoantibodies in dermatomyositis-associated interstitial lung disease and establishment of treatment for improved life prognosis
| Project/Area Number |
16H05341
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Collagenous pathology/Allergology
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| Research Institution | Kyoto University |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
OMURA Koichiro 京都大学, 大学院医学研究科, 准教授 (40432372)
YOSHIFUJI Hajime 京都大学, 大学院医学研究科, 助教 (20422975)
IMURA Yoshitaka 京都大学, 大学院医学研究科, 助教 (60456895)
NAKASHIMA Ran 京都大学, 大学院医学研究科, 助教 (10599525)
MURAKAMI Kousaku 京都大学, 大学院医学研究科, 助教 (70599927)
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| Research Collaborator |
HOSONO Yuji
TSUJI Hideki
YAGITA Nasato
HATTA Kazuhiro
NOJIMA Takaki
KATAYAMA Masaki
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| Project Period (FY) |
2016-04-01 – 2019-03-31
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| Keywords | MDA5 / IFIH1 / SFPQ / 自己抗体 / 間質性肺炎 / 皮膚筋炎 / 免疫抑制療法 / 血漿交換療法 |
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| Outline of Final Research Achievements |
Anti-MDA5 antibody is a specific marker for intractable dermatomyositis (DM) with interstitial lung disease (ILD). We found a novel antibody in patients with anti-MDA5 and identified the target antigen as SFPQ.
To improve the life prognosis of anti-MDA5-positive patients, we conducted a multicenter prospective study to confirm efficacy and safety of the combined immunosuppressive drugs containing high dose glucocorticoid, oral tacrolimus and intravenous cyclophosphamide. Finally, 29 patients with anti-MDA5-positive ILD-DM/CDAM were registered, and the 6 month survival rate as the primary endpoint was 89%, which was significantly higher than that of historical control (33%). Moreover, we found that plasmapheresis was effective for immunosuppressive drug-resistant cases.
Thus, we demonstrate the efficacy of combined immunosuppressive drugs and plasmapheresis against intractable anti-MDA5-positive ILD-DM/CDAM.
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| Free Research Field |
臨床免疫学
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| Academic Significance and Societal Importance of the Research Achievements |
抗MDA5抗体は難治性で死亡率の高い急速進行性間質性肺病変（ILD）を合併する皮膚筋炎（DM）と無筋症性皮膚筋炎（CADM）に特異的に検出される自己抗体である。我々は同疾患の生命予後改善を目的とする多施設共同前向き臨床研究を遂行し、ステロイド大量、タクロリムス、シクロホスファミド間歇静注療法の同時併用療法が有効であることを証明した。さらに、同療法に抵抗性を示す症例に血漿交換療法を併用することでさらなる生命予後の改善を確認した。これらの成績より、有効な治療法が確立されていなかった膠原病の難治性病態の一つであるILD合併DM/CADMのスタンダードな治療法を確立できたものと考える。
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