The role of immunosuppressive receptor LILRB in the pathogenesis of Kawasaki disease, and its application to the therapy.

2016 Fiscal Year Final Research Report

• Project/Area Number: 26461597
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Pediatrics
• Research Institution: Department of Clinical Research, National Hospital Organization Sendai Medical Center
• Principal Investigator: Kumaki Satoru 独立行政法人国立病院機構(仙台医療センター臨床研究部), その他部局等, 部長 (20311566)
• Project Period (FY): 2014-04-01 – 2017-03-31
• Keywords: 川崎病 / 免疫抑制性受容体 / LILRB4 / 免疫グロブリン大量療法 / plasmablast

Outline of Final Research Achievements

Kawasaki disease (KD) is an acute vasculitis of unknown pathogenesis which occurs most frequently in early childhood, and intravenous immunoglobulin (IVIG) is effective. To obtain a mechanistic insight into the humoral immune response in KD patients, B cell subpopulations and the expression pattern of immunosuppressive receptor LILRB was analyzed. As a result, proportion of memory B cells increased in acute phase of the KD patients when compared with normal controls. Furthermore, the proportion of plasmablasts was significantly elevated in the acute phase of KD, which returned to the basal level after IVIG. From these results, it was suggested that adaptive immunity is involved in the pathogenesis of KD. In addition, we demonstrate that the expression of LILRB4 was enhanced in the acute phase of KD, and the expression was controlled by IVIG. These results suggest that plasmablasts with increased LILRB4 expression play an important role in the pathogenesis of KD.

Free Research Field

小児科