Abstract
Background: Kawasaki disease is the most prevalent vasculitis of children in the developed countries that affects middle-sized arteries. Though T-cells are known to be activated with ample production of cytokines in acute phase of Kawasaki disease, there is a paucity of data concerning dendritic cells (DCs), the most potent antigen presenting cells that initiates T-cell activation. This study examined change in circulating DCs in acute phase of Kawasaki disease. Methods: Using multi-color flow cytometry, we determined circulating myeloid DC (mDC), Lin-HLA-DR+CD11c+ cell, and plasmacytoid DC (pDC), Lin-HLA-DR+CD123+ cell in 33 patients with acute phase of Kawasaki disease (aKD), 24 febrile controls (FC), and 13 healthy controls (HC). Blood chemistry data including cytokines were determined at the same time. Numbers of DCs were compared among 3 groups and before and after immunoglobulin treatment in aKD. Correlation between numbers of circulating DCs and blood chemistry data were determined. Results: Number of circulating mDC was significantly lower in aKD on admission than in FC and HC [median (lower, upper quartile)=7260 (2463, 11550) vs. 12210 (9500, 22050) and 18600 (11520, 23460) cells/ml, p < 0.001]. This number of circulating DCs significantly correlated with disease severity represented by serum albumin (mDC, r=0.56, p < 0.0001; pDC, r=0.39, p < 0.02, respectively), C reactive protein (mDC, r=-0.42, p < 0.005), and interleukin-6 (mDC, r=-0.55, p < 0.007). Immunoglobulin treatment quickly restored number of mDC [7260 (2463, 11550) vs. 15200 (10840, 30965) after IVIG and 18600 (12950, 25510) cells/ml at convalescence, p < 0.001] in aKD. Conclusions: This study indicates that number of circulating mDCs is decreased in acute Kawasaki disease, and may be involved in the pathophysiology.
Highlights
Kawasaki disease is the most prevalent systemic vasculitis, and affects middle-sized arteries including coronary arteries, of children in developed countries [1,2]
There was no significant difference in age or sex distribution among 3 groups and number of febrile days at study between acute phase of Kawasaki disease (aKD) and febrile controls (FC)
There was no significant difference between aKD and FC in white blood cell counts, C-reactive protein (CRP), hematocrit, and sodium
Summary
Kawasaki disease is the most prevalent systemic vasculitis, and affects middle-sized arteries including coronary arteries, of children in developed countries [1,2]. Myeloid DC express CD11c, leukocyte integrin alpha subunit, and polarize naïve T cells toward the T-helper 1 (Th1) phenotype, whereas pDC express CD123, interleukin-3 receptor alpha chain, and polarize T cells toward the Th2 phenotype. These DC subsets recognize different microbial pathogens through specific receptors, which in turn induce different types of innate and adaptive immune responses [10]. Though T-cells are known to be activated with ample production of cytokines in acute phase of Kawasaki disease, there is a paucity of data concerning dendritic cells (DCs), the most potent antigen presenting cells that initiates T-cell activation. This study examined change in circulating DCs in acute phase of Kawasaki disease
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