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Clinical epidemiology of MGRS-associated kidney diseases

Research Project

Project/Area Number 20K08600
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Review Section Basic Section 53040:Nephrology-related
Research InstitutionFujita Health University

Principal Investigator

HAYASHI Hiroki  藤田医科大学, 医学部, 准教授 (10378086)

Co-Investigator(Kenkyū-buntansha) 湯澤 由紀夫  藤田医科大学, 医学部, 教授 (00191479)
尾形 宗士郎  国立研究開発法人国立循環器病研究センター, 研究所, 室長 (00805012)
坪井 直毅  藤田医科大学, 医学部, 教授 (50566958)
稲熊 大城  藤田医科大学, 医学部, 教授 (60791069)
冨田 章裕  藤田医科大学, 医学部, 教授 (80378215)
高橋 和男  藤田医科大学, 医学部, 教授 (90631391)
Project Period (FY) 2020-04-01 – 2023-03-31
Project Status Completed (Fiscal Year 2022)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2022: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2021: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2020: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
KeywordsMGRS / M蛋白血症 / Clone Directed Therapy / 臨床疫学 / M蛋白関連腎症
Outline of Research at the Start

M蛋白関連腎症のうち, 血液悪性腫瘍の診断および介入基準を満たさない血液疾患群をmonoclonal gammopathy of renal significance(MGRS)と総称することが提唱され, MGRS関連腎症の病理学的知見は蓄積したが, 稀少性から臨床疫学データは皆無である. 本研究ではMGRS関連腎症の臨床・病理像を横断的かつ縦断的に検証し, MGRSとしてカテゴライズすることの臨床的意義, および血液悪性腫瘍に準じM蛋白に早期より積極的介入することの重要性を発信する.

Outline of Final Research Achievements

Although the term MGRS has long been proposed for a group of hematologic diseases with M-protein-associated nephropathy that do not meet the diagnostic and interventional criteria for hematologic malignancies, clinical epidemiological data are limited due to the rarity of the disease. We established a cohort of patients with M-protein-associated nephropathy and examined the pathological and clinical features of MGRS cross-sectionally and longitudinally, using malignant B-cell tumors/ malignant plasma cell tumors (BCM/PCM) as controls.
Typical cases of BCM/PCM showed acute renal symptoms and cast nephropathy with "M protein quantity" problem, while typical cases of MGRS showed chronic renal symptoms and various glomerular lesions with "M protein quality" problem. Compared to BCM/PCM, MGRS patients were less frequently treated with Clone Directed Therapy, resulting in a less favorable renal and overall prognosis.

Academic Significance and Societal Importance of the Research Achievements

単クローン性の免疫グロブリン(M蛋白)が関与するM蛋白関連腎症のうち, 血液悪性腫瘍の診断および治療介入基準を満たさない血液疾患群をMGRSと総称することが提唱されて久しいが, その稀少性から臨床疫学データは皆無である. 我々はこれまでに報告の無いMGRSならびにMGRS関連腎症の臨床疫学データを, 自施設のコホートと我が国の腎生検レジストリーから, 世界に先駆け明らかにした. 本研究は, これまでは疾患概念にとどまったMGRSを, 「各科の密な連携により, 早期診断・早期介入することが重要な予後不良な疾患群である」と, 認識をあらためるよう啓発する責務を負う研究であると位置づけている.

Report

(4 results)
  • 2022 Annual Research Report   Final Research Report ( PDF )
  • 2021 Research-status Report
  • 2020 Research-status Report
  • Research Products

    (8 results)

All 2022 2021 2020

All Journal Article (4 results) (of which Peer Reviewed: 3 results,  Open Access: 3 results) Presentation (4 results) (of which Invited: 1 results)

  • [Journal Article] 【腎臓医が診る指定難病】指定難病各論 膠原病関連腎疾患 シェーグレン症候群2021

    • Author(s)
      梅田 良祐, 林 宏樹, 湯澤 由紀夫
    • Journal Title

      腎と透析

      Volume: 91 Pages: 143-148

    • Related Report
      2021 Research-status Report
  • [Journal Article] Long-term changes in renal function after treatment initiation and the importance of early diagnosis in maintaining renal function among IgG4-related tubulointerstitial nephritis patients in Japan2020

    • Author(s)
      Arai Haruna、Ogata Soshiro、Ozeki Takaya、Takahashi Kazuo、Tsuboi Naotake、Maruyama Shoichi、Inaguma Daijo、Hasegawa Midori、Yuzawa Yukio、Hayashi Hiroki
    • Journal Title

      Arthritis Research & Therapy

      Volume: 22 Issue: 1

    • DOI

      10.1186/s13075-020-02320-x

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Comparison of the 2018 and 2003 International Society of Nephrology/Renal Pathology Society classification in terms of renal prognosis in patients of lupus nephritis: a retrospective cohort study2020

    • Author(s)
      Umeda Ryosuke、Ogata Soshiro、Hara Shigeo、Takahashi Kazuo、Inaguma Daijo、Hasegawa Midori、Yasuoka Hidetaka、Yuzawa Yukio、Hayashi Hiroki、Tsuboi Naotake
    • Journal Title

      Arthritis Research & Therapy

      Volume: 22 Issue: 1

    • DOI

      10.1186/s13075-020-02358-x

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Better remission rates in elderly Japanese patients with primary membranous nephropathy in nationwide real-world practice: The Japan Nephrotic Syndrome Cohort Study (JNSCS)2020

    • Author(s)
      Yokoyama Hitoshi、Yamamoto Ryohei、Imai Enyu、Maruyama Shoichi、Sugiyama Hitoshi、et al.
    • Journal Title

      Clinical and Experimental Nephrology

      Volume: 24 Issue: 10 Pages: 893

    • DOI

      10.1007/s10157-020-01913-9

    • URL

      https://pure.teikyo.jp/en/publications/6c591462-83b8-4342-bee4-6e86c6f9dfa2

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] Monoclonal immunoglobulin(MIg)関連腎臓病の治療介入は十分か?2022

    • Author(s)
      成宮利幸, 林宏樹, 尾形宗士郎, 高橋和男, 小出滋久, 稲熊大城, 長谷川みどり, 湯澤由紀夫, 坪井直毅, 原重雄
    • Organizer
      第65回日本腎臓学会学術総会
    • Related Report
      2022 Annual Research Report
  • [Presentation] Monoclonal gammopathy 関連腎疾患の予後2021

    • Author(s)
      成宮利幸,尾形宗士郎,高橋和男,林 宏樹,小出滋 久,稲熊大城,長谷川みどり,湯澤由紀夫,坪井直毅
    • Organizer
      第64 回日本腎臓学会学術総会
    • Related Report
      2021 Research-status Report
  • [Presentation] 二次性糸球体疾患の基礎と臨床:ループス腎炎の改訂組織分類と新規バイオマーカー2020

    • Author(s)
      坪井直毅,林 宏樹
    • Organizer
      第63回日本腎臓学会学術総会
    • Related Report
      2020 Research-status Report
    • Invited
  • [Presentation] IgG4関連TINの腎生検による診断率と長期腎予後の検討2020

    • Author(s)
      新居春菜、尾形宗士郎、尾関貴哉、林宏樹、高橋和男、小出滋久、坪井直毅、丸山彰一、稲熊大城、長谷川みどり、湯澤由紀夫
    • Organizer
      第63回日本腎臓学会学術総会
    • Related Report
      2020 Research-status Report

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Published: 2020-04-28   Modified: 2024-01-30  

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