Elucidation of the mechanisms of orang fibrosis using patients with IgG4-related disease and their mouse models

2022 Fiscal Year Final Research Report

• Project/Area Number: 19H03854
• Research Category: Grant-in-Aid for Scientific Research (B)
• Allocation Type: Single-year Grants
• Section: 一般
• Review Section: Basic Section 57060:Surgical dentistry-related
• Research Institution: Kyushu University
• Principal Investigator: Maehara Takashi 九州大学, 歯学研究院, 助教 (10637333)
• Co-Investigator(Kenkyū-buntansha): 新納 宏昭 九州大学, 医学研究院, 教授 (20380636) ; 森山 雅文 九州大学, 大学病院, 助教 (20452774) ; 山元 英崇 九州大学, 大学病院, 准教授 (30404073) ; 中村 誠司 九州大学, 歯学研究院, 教授 (60189040)
• Project Period (FY): 2019-04-01 – 2023-03-31
• Keywords: IgG4関連疾患 / 全身性強皮症 / 線維化

Outline of Final Research Achievements

IgG4-related diseases, which were first proposed in Japan and are now attracting worldwide attention, are diseases characterized by lesions in organs throughout the body and irreversible organ fibrosis associated with infiltration of T and B cells into the affected organs. However, the molecular mechanism of organ fibrosis that characterizes this disease is still unknown, and the disease is intractable with no effective treatment. Because fibrosis is irreversible and leads to deterioration of organ function, the development of novel therapeutic methods is required. In this study, we identified lymphocytes involved in organ fibrosis and investigated their relationship with characteristic macrophages involved in organ fibrosis.

Free Research Field

臨床免疫学

Academic Significance and Societal Importance of the Research Achievements

IgG4-RD は本邦から提唱された疾患で、涙腺・唾液腺炎を初めとして自己免疫性膵炎、後腹膜線維症、硬化性胆管炎、間質性肺炎などの多くの病変を生じ、活性化した T 細胞と B 細胞の組織浸潤や特徴的なマクロファージの浸潤に伴う不可逆性の臓器線維化を特徴とする全身疾患である。この臓器線維化のメカニズムを明らかにすることができれば、新たな治療標的分子を明らかにすることができる。