Dr. S.-J. Lee and Ms Y.-N. Cho contributed equally to this work.
Natural killer T cell deficiency in active adult-onset Still's disease: Correlation of deficiency of natural killer T cells with dysfunction of natural killer cells
Article first published online: 27 AUG 2012
Copyright © 2012 by the American College of Rheumatology
Arthritis & Rheumatism
Volume 64, Issue 9, pages 2868–2877, September 2012
How to Cite
Lee, S.-J., Cho, Y.-N., Kim, T.-J., Park, S.-C., Park, D.-J., Jin, H.-M., Lee, S.-S., Kee, S.-J., Kim, N., Yoo, D.-H. and Park, Y.-W. (2012), Natural killer T cell deficiency in active adult-onset Still's disease: Correlation of deficiency of natural killer T cells with dysfunction of natural killer cells. Arthritis & Rheumatism, 64: 2868–2877. doi: 10.1002/art.34514
- Issue published online: 27 AUG 2012
- Article first published online: 27 AUG 2012
- Accepted manuscript online: 17 MAY 2012 02:30PM EST
- Manuscript Accepted: 17 APR 2012
- Manuscript Received: 5 AUG 2011
- National Research Foundation of Korea. Grant Number: 2011-0011332
- Korean government
- Chonnam National University Hospital Research Institute of Clinical Medicine. Grant Number: CRI12057-21
- Korean Ministry for Health, Welfare, and Family Affairs. Grant Numbers: Health Technology R&D Projects A100004, A110397
To examine the levels and functions of natural killer (NK) and natural killer T (NKT) cells, investigate relationships between NK and NKT cells, and determine the clinical relevance of NKT cell levels in patients with adult-onset Still's disease (AOSD).
Patients with active untreated AOSD (n = 20) and age- and sex-matched healthy controls (n = 20) were studied. NK and NKT cell levels were measured by flow cytometry. Peripheral blood mononuclear cells were cultured in vitro with α-galactosylceramide (αGalCer). NK cytotoxicity against K562 cells and proliferation indices of NKT cells were estimated by flow cytometry.
Percentages and absolute numbers of NKT cells were significantly lower in the peripheral blood of AOSD patients than in that of healthy controls. Proliferative responses of NKT cells to αGalCer were also lower in patients, and this was found to be due to proinflammatory cytokines and NKT cell apoptosis. In addition, NK cytotoxicity was found to be significantly lower in patients than in healthy controls, but NK cell levels were comparable in the 2 groups. Notably, this NKT cell deficiency was found to be correlated with NK cell dysfunction and to reflect active disease status. Furthermore, αGalCer-mediated NK cytotoxicity, showing the interaction between NK and NKT cells, was significantly lower in AOSD patients than in healthy controls.
These findings demonstrate that NK and NKT cell functions are defective in AOSD patients and suggest that these abnormalities contribute to innate immune dysfunction in AOSD.