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Updated: Jul 20, 2026
Single-cell Analysis of Immunophenotype and Cytokine Production in Peripheral Whole Blood via Mass Cytometry
Published on: June 26, 2018
Expression of myeloid-related protein-8 and -14 in patients with acute Kawasaki disease
Keiich Hirono1, Dirk Foell, Yanlin Xing
1Department of Pediatrics, Toyama University, Toyama, Japan.
Objectives:
This study investigated patients with acute Kawasaki disease (KD) to validate myeloid-related protein (MRP)-8/MRP-14 as a marker of disease activity and severity of coronary artery lesion development.
Background:
Both MRP-8 and -14, which are S100-proteins secreted by activated neutrophils and monocytes, bind specifically to endothelial cells and induce thrombogenic and inflammatory responses in a variety of disease conditions.
Methods:
We investigated 61 patients with acute KD and examined sequential changes in serum levels of MRP-8/MRP-14, messenger ribonucleic acid (mRNA) expression of MRP-8 and -14 in circulating granulocytes and monocytes, and amounts of MRP-8/MRP-14 bound to circulating endothelial cells.
Results:
The serum MRP-8/MRP-14 levels as well as mRNA expressions of MRP-8 and -14 in granulocytes were strongly upregulated during the early stage of acute KD, and decreased dramatically within 24 h of intravenous immune globulin therapy (p < 0.05) in 45 responders. In contrast, in 16 nonresponders both of these increased after the initial treatment. The number of MRP-8/MRP-14-positive circulating endothelial cells was higher in patients with acute KD than in control patients and increased significantly by 2 weeks after the onset of KD, especially in patients in whom coronary artery lesions developed.
Conclusions:
We show for the first time that MRP-8/MRP-14 are exclusively secreted by granulocytes in patients with acute KD, and intravenous immune globulin treatment suppresses their gene expression. Serum levels of MRP-8/MRP-14 may be useful markers of disease activity, and the levels of MRP-8/MRP-14-positive circulating endothelial cell may predict the severity of vasculitis, confirming an important role for distinct inflammatory reactions in endothelium.
Insights
Myeloid-related protein (MRP)-8/MRP-14 levels indicate Kawasaki disease activity. Elevated MRP-8/MRP-14 in circulating endothelial cells may predict coronary artery lesion severity in children.
Area of Science:
- Immunology
- Pediatric Rheumatology
- Biomarker Discovery
Background:
- Myeloid-related proteins (MRP)-8 and -14 are S100-proteins released by neutrophils and monocytes.
- These proteins interact with endothelial cells, promoting inflammation and thrombosis in various conditions.
Purpose of the Study:
- To evaluate myeloid-related protein (MRP)-8/MRP-14 as a biomarker for Kawasaki disease (KD) activity.
- To assess the correlation between MRP-8/MRP-14 levels and the development of coronary artery lesions in KD patients.
Main Methods:
- Serum levels and mRNA expression of MRP-8/MRP-14 were analyzed in 61 acute KD patients.
- Levels of MRP-8/MRP-14 bound to circulating endothelial cells were quantified.
- Changes were monitored following intravenous immune globulin (IVIG) therapy.
Main Results:
- MRP-8/MRP-14 levels and granulocyte mRNA expression were elevated in acute KD, decreasing after IVIG in responders.
- Non-responders showed increased levels post-treatment.
- Elevated MRP-8/MRP-14-positive circulating endothelial cells correlated with KD and coronary artery lesion development.
Conclusions:
- MRP-8/MRP-14 are primarily secreted by granulocytes in acute KD.
- IVIG treatment suppresses MRP gene expression.
- Serum MRP-8/MRP-14 levels may indicate disease activity, while circulating endothelial cell-bound MRP-8/MRP-14 could predict vasculitis severity.