| Project/Area Number |
18K08419
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Review Section |
Basic Section 54020:Connective tissue disease and allergy-related
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| Research Institution | Tokai University |
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Principal Investigator |
Sato Shinji 東海大学, 医学部, 教授 (90276238)
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| Project Period (FY) |
2018-04-01 – 2021-03-31
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| Project Status |
Completed (Fiscal Year 2020)
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| Budget Amount *help |
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2020: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2019: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2018: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
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| Keywords | 抗MDA5抗体 / 皮膚筋炎 / 急速進行性間質性肺炎 / 予後予測 / 新規治療 / 自己抗体 / 間質性肺疾患 / 予後不良因子 / 血漿交換療法 / 間質性肺炎 / 免疫学 / 無筋症性皮膚筋炎 |
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| Outline of Final Research Achievements |
Anti-Melanoma Differentiation-Associated Gene 5 (MDA5) antibody is one of DM-specific autoantibodies and is well known by its close association with RP-ILD. Previous studies revealed that anti-MDA5 antibody is a powerful predictive factor for poor prognosis. Other than anti-MDA5 antibody positivity, several risk factors for poor prognosis, such as age ≧60 years, SpO2< 95%, CRP≧ 1mg/dL，ferritin≧ 500ng/dL，KL-6≧ 1,000 U/dL were reported. This study suggested that high concentration of serum SP-D was also a predictive factor for poor prognosis. As for treatment for refractory cases of DM and RP-ILD, we found that the early initiation of plasma exchange therapy in addition to intensive immunosuppressive combination therapy improved the prognosis of this condition. Our findings also highlight the usefulness of changes in anti-MDA5 antibody titer as well as serum ferritin or KL-6 levels in monitoring of disease activity and prediction of disease outcome.
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| Academic Significance and Societal Importance of the Research Achievements |
DMに併発するRP-ILDは，急速に進行する抵抗性の予後不良の病態であるが，早期に診断し，可能な限り早期より強力な治療を開始することで救命も可能であることがあきらかになっている．近年，単アーム多施設前向き試験で副腎皮質ステロイド大量療法に免疫抑制薬多剤併用の有効性が示されたが，この治療を行っても，治療開始時に症状が進行している症例を中心に治療が奏功しないあるいは重症な感染症を合併して不幸な結果に終わる症例が未だ多く存在する．かかる点において本研究の成果は，DM/RP-ILD予後不良予測因子のさらなる追究，生存率をより向上させる新規治療法の開発という点から学術的・社会的意義あると考えられる．
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