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Search for progression factors and novel therapeutic targets in igG4-related lung disease

Research Project

Project/Area Number 15K09212
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Respiratory organ internal medicine
Research InstitutionUniversity of Toyama

Principal Investigator

Matsui Shoko  富山大学, 保健管理センター, 教授 (40334726)

Co-Investigator(Kenkyū-buntansha) 伊藤 清亮  金沢大学, 附属病院, 医員 (10467110)
川野 充弘  金沢大学, 附属病院, 講師 (20361983)
林 龍二  富山大学, 附属病院, 教授 (60345585)
山田 和徳  金沢大学, 医薬保健学総合研究科, 特任准教授 (90397224)
Project Period (FY) 2015-04-01 – 2019-03-31
Project Status Completed (Fiscal Year 2018)
Budget Amount *help
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2017: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2016: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
KeywordsIgG4関連肺疾患 / 自然免疫 / モデルマウス / IgG4関連疾患 / IgG4関連肺疾患 / 免疫学
Outline of Final Research Achievements

IgG4-related disease (IgG4-RD) is a recently recognized systemic disease, characterized by tumefactive lesions with abundant IgG4-positive plasma cell infiltrates and elevated serum IgG4 concentrations. Although systemic corticosteroid therapy is effective, there is often recurrence of the lesion in IgG4-RD. We aimed to establish model mice of IgG4-RD and evaluated that the phenotypes of LatY136F knock-in mice form a pathological condition similar to IgG4-related lung disease. We also studied the involvement of innate immunity and its control in the lung lesions.
As a result, we found that innate immunity may be involved in the pathogenesis of IgG4-related lung disease and got clues for treatment other than glucocorticoids.

Academic Significance and Societal Importance of the Research Achievements

IgG4 関連肺疾患発症および進展に関与する因子が同定されれば、病態の解明や診断や新規治療のターゲットとなり得ると考え、外界から刺激をうける呼吸器の自然免疫に着目した。その結果、モデルマウスを用いて自然免疫の関与を確認したことから、新たな治療法の手がかりを得ることができ、今後の研究の発展が望めると考えられた。

Report

(5 results)
  • 2018 Annual Research Report   Final Research Report ( PDF )
  • 2017 Research-status Report
  • 2016 Research-status Report
  • 2015 Research-status Report
  • Research Products

    (20 results)

All 2018 2017 2016 2015

All Journal Article (8 results) (of which Int'l Joint Research: 1 results,  Peer Reviewed: 4 results,  Open Access: 3 results) Presentation (12 results) (of which Int'l Joint Research: 8 results,  Invited: 1 results)

  • [Journal Article] Comparison of clinical and pathological features of lung lesions of systemic IgG4-related disease and idiopathic multicentric Castleman’s disease.2017

    • Author(s)
      Terasaki Y, Ikushima S, Matsui S, et.al.
    • Journal Title

      Histopathology

      Volume: 印刷中

    • Related Report
      2016 Research-status Report
    • Peer Reviewed
  • [Journal Article] Proposed diagnostic criteria for IgG4-related respiratory disease2016

    • Author(s)
      Matsui S, Yamamoto H, Minamoto S, et.al.
    • Journal Title

      Respir Invest

      Volume: 54 Pages: 130-2

    • Related Report
      2016 Research-status Report 2015 Research-status Report
  • [Journal Article] Factors related to renal cortical atrophy development after glucocorticoid therapy in IgG4-related kidney disease: a retrospective multicenter study2016

    • Author(s)
      Mizushima I, Yamamoto M, Inoue D, et al.
    • Journal Title

      Arthritis Res Ther

      Volume: 18 Pages: 273-273

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] IgG4関連疾患の病因・病態を考える-IgG4関連呼吸器疾患から2016

    • Author(s)
      松井祥子
    • Journal Title

      分子リウマチ治療

      Volume: 9 Pages: 13-16

    • Related Report
      2016 Research-status Report
  • [Journal Article] IgG4関連呼吸器疾患の新知見2016

    • Author(s)
      2)松井祥子
    • Journal Title

      医学のあゆみ

      Volume: 258 Pages: 241-4

    • Related Report
      2016 Research-status Report
  • [Journal Article] Decreased Expression of Innate Immunity-Related Genes in Peripheral Blood Mononuclear Cells from Patients with IgG4-Related Disease2015

    • Author(s)
      Nakajima A, Masaki Y, Nakamura T,et al.
    • Journal Title

      Pros One

      Volume: 10 Issue: 5 Pages: e0126582-e0126582

    • DOI

      10.1371/journal.pone.0126582

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] International consensus guidance statement on the management and treatment of IgG4-related disease2015

    • Author(s)
      Khosroshahi A, Wallace ZS, Crowe JL, Kawa S, Stone JH.et al.
    • Journal Title

      Arthritis Rheumatol.

      Volume: 67 Issue: 7 Pages: 1688

    • DOI

      10.1002/art.39132

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] IgG4関連呼吸器疾患2015

    • Author(s)
      松井祥子
    • Journal Title

      Modern Physician

      Volume: 35 Pages: 1339-42

    • Related Report
      2015 Research-status Report
  • [Presentation] Evaluation of Lung Lesions in LATY136F Mutant Mice.2018

    • Author(s)
      Waseda Y, Matsui S, Yamada K, Mizuguchi K, Watanabe S, Ito K, Zuka M, Malissen M, Kawano M, Ishizuka T.
    • Organizer
      ATS 2018 International Conference
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Lat Y136F knock-inマウス(IgG4関連肺疾患モデルマウス)の肺病変の解析2018

    • Author(s)
      早稲田優子,松井祥子,渡辺知志,佐藤譲之,杉山光寿,中嶋康貴,三ツ井美穂,島田昭和,園田智明,山口牧子,本定千知,門脇麻衣子,重見博子,梅田幸寛,森川美羽,安斎正樹,石塚全.
    • Organizer
      第58回日本呼吸器学会学術講演会
    • Related Report
      2018 Annual Research Report
  • [Presentation] Allergy in IgG4-related disease2018

    • Author(s)
      Matsui S, Okazawa S, Tokui K, Kambara K, Imanishi S, Taka C, Yamada T, Inomata M, Miwa T, Hayashi R
    • Organizer
      AAAAI/WAOJoint Congress 2018
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research
  • [Presentation] IgG4-related respiratory disease and its mimicskers.2017

    • Author(s)
      Matsui S, Minamoto S, Yamamoto H, Handa T, Waseda Y, Mishima M, Kubo K.
    • Organizer
      The 3rd International Symposium on IgG4-RD and Fibrosis.
    • Place of Presentation
      Hawaii, USA
    • Year and Date
      2017-02-15
    • Related Report
      2016 Research-status Report
    • Int'l Joint Research
  • [Presentation] Serum soluble IL-2 receptor as a biomarker in IgG4 related disease.2017

    • Author(s)
      Handa T, Matsui S, Yoshifuji H, Tanizawa K, Kodama Y, Yamamoto H, Minamoto S, Waseda Y, Ikezoe K, Chin K, Hirai T, Kubo K, Mimori T, Chiba T, Mishima M.
    • Organizer
      The 3rd International Symposium on IgG4-RD and Fibrosis.
    • Place of Presentation
      Hawaii, USA
    • Year and Date
      2017-02-15
    • Related Report
      2016 Research-status Report
    • Int'l Joint Research
  • [Presentation] IgG4-related respiratory disease and its mimickers2017

    • Author(s)
      1) Matsui S, Yamamoto H, Handa T, Minamoto S, Waseda Y, Mishima M, Kubo K
    • Organizer
      The 3rd International Symposium on IgG4-RD & fibrosis
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research
  • [Presentation] IgG4-related respiratory disease and its mimickers2017

    • Author(s)
      4) Matsui S, Yamamoto H, Handa T, Minamoto S, Waseda Y, Mishima M, Kubo K
    • Organizer
      ATS 2017 International Conference
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research
  • [Presentation] Clinical features of IgG4-related Respiratory Disease.2016

    • Author(s)
      Matsui S, Yamamoto H, Handa T, Minamoto S, Waseda Y, Mishima M, Kubo K.
    • Organizer
      ATS 2016 International Conference
    • Place of Presentation
      San Francisco
    • Year and Date
      2016-05-13
    • Related Report
      2016 Research-status Report
    • Int'l Joint Research
  • [Presentation] IgG4関連疾患における抗酸菌感染の検討.2015

    • Author(s)
      松井祥子,林 龍二,濱島 丈,他
    • Organizer
      第24回日本シェーグレン症候群学会学術集会
    • Place of Presentation
      東京
    • Year and Date
      2015-09-18
    • Related Report
      2015 Research-status Report
  • [Presentation] Proposal for diagnostic criteria for IgG4-related respiratory disease2015

    • Author(s)
      Matsui S, Yamamoto H, Handa T,et al.
    • Organizer
      ATS 2015 International Conference
    • Place of Presentation
      Denver
    • Year and Date
      2015-05-15
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] IgG4関連肺疾患モデルマウスの確立 -Lat Y136F knock-inマウスの肺病変の解析-2015

    • Author(s)
      早稲田優子, 松井祥子, 佐伯啓吾 他
    • Organizer
      第55回日本呼吸器学会学術講演会
    • Place of Presentation
      東京
    • Year and Date
      2015-04-17
    • Related Report
      2015 Research-status Report
  • [Presentation] IgG4関連疾患における最近の進歩~IgG4関連呼吸器疾患2015

    • Author(s)
      久保惠嗣, 松井祥子
    • Organizer
      第112回日本内科学会講演会
    • Place of Presentation
      京都
    • Year and Date
      2015-04-10
    • Related Report
      2015 Research-status Report
    • Invited

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Published: 2015-04-16   Modified: 2020-03-30  

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