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

2018 Fiscal Year Final Research Report

• Project/Area Number: 15K09212
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Respiratory organ internal medicine
• Research Institution: University 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
• Keywords: ＩｇＧ４関連肺疾患 / 自然免疫 / モデルマウス

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.

Free Research Field

呼吸器内科学

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。