PT - JOURNAL ARTICLE AU - Yasuhiro Shimojima AU - Dai Kishida AU - Ken-ichi Ueno AU - Satoru Ushiyama AU - Takanori Ichikawa AU - Yoshiki Sekijima TI - Characteristics of Circulating Natural Killer Cells and Their Interferon-γ Production in Active Adult-onset Still Disease AID - 10.3899/jrheum.181192 DP - 2019 Feb 15 TA - The Journal of Rheumatology PG - jrheum.181192 4099 - http://www.jrheum.org/content/early/2019/06/11/jrheum.181192.short 4100 - http://www.jrheum.org/content/early/2019/06/11/jrheum.181192.full AB - Objective To investigate the characteristics of circulating natural killer (NK) cells and their interferon (IFN)-γ–producing ability in adult-onset Still disease (AOSD). Methods Peripheral blood mononuclear cells were obtained from 22 patients in the acute phase of AOSD (acute AOSD); 7 of the 22 patients after treatment (remission AOSD), and 11 healthy controls (HC). NK cells and their IFN-γ expression levels were analyzed by flow cytometry. Additionally, the cytokine receptors of interleukin (IL)-12, IL-15, and IL-18 on NK cells were also evaluated. Results The frequency of NK cells was significantly lower in acute AOSD than in HC. NK cell counts significantly increased in remission AOSD. Expression of IL-12 and IL-15 receptors on NK cells was significantly increased in acute AOSD, whereas that of IL-18 receptor indicated no significant difference among 3 groups. IFN-γ expression in NK cells was significantly higher in acute AOSD than in HC, and significantly decreased in remission AOSD. The absolute number of NK cells and IFN-γ–expressing NK cells revealed an inverse correlation with serum ferritin levels in acute AOSD. In 2 distinct subsets of NK cells, CD56dim NK cells significantly exhibited higher IFN-γ expression than CD56bright NK cells in acute AOSD. Conclusion In acute AOSD, NK cells displayed lower proportion, whereas they had higher ability for IFN-γ production than in HC; moreover, upregulation of IL-12 and IL-15 receptors on NK cells may promote IFN-γ production. In addition, disease activity may be implicated in regulating the number of NK cells and IFN-γ–expressing NK cells in AOSD.