Clarification of pathophysiology of macrophage activation syndrome in adult Still disease

2023 Fiscal Year Final Research Report

• Project/Area Number: 21K08479
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 54020:Connective tissue disease and allergy-related
• Research Institution: Keio University
• Principal Investigator: Kaneko Yuko 慶應義塾大学, 医学部(信濃町), 教授 (60317112)
• Project Period (FY): 2021-04-01 – 2024-03-31
• Keywords: 成人スティル病 / マクロファージ

Outline of Final Research Achievements

Gene expression was examined in the peripheral blood of 72 adult Still's disease patients and 16 healthy subjects attending the applicant's hospital. The results suggest that the disease activity may significantly reflect the disease activity in the validation cohort. A total of 37 CDs and cell surface globulins were stained by mass cytometry in the peripheral blood of 37 adult Still's disease patients, 5 healthy subjects, and 60 patients with other collagen diseases, and 47 clusters characteristic of adult Still's disease patients were identified. A characteristic residual number of cells, mainly NK cells, were found in adult Still's disease patients in remission, suggesting that they play a role in the macrophage activation mechanism.

Free Research Field

臨床免疫学

Academic Significance and Societal Importance of the Research Achievements

成人スティル病は発熱、関節炎、皮疹を三主徴とする原因不明の炎症性疾患である。病態の主体は、マクロファージや好中球を主体とした自然免疫系の異常活性化が病態と考えられている。マクロファージ活性化症候群は、成人スティル病に併発する致死的重症病態であるが、疾患活動期とマクロファージ活性化症候群併発時のマクロファージ活性化の相違は明確でなかった。本研究で発現遺伝子パターンおよび活性化免疫細胞の詳細な検討を通じて、マクロファージ活性化の定量化と病態に重要な細胞を同定した意義は大きく、さらなる検討によりマクロファージ活性化発症予測や適切な治療介入につながる可能性がある。