Department of Pediatrics, University of California San Diego School of Medicine and Rady Children's Hospital, La Jolla, CA 92093-0641, USA.
Autoimmunity. 2010 Jun;43(4):317-24. doi: 10.3109/08916930903405891.
Kawasaki disease (KD) is a pediatric self-limited vasculitis characterized by immune-mediated destruction of the arterial wall and myocardium. Neither the trigger that incites the inflammation nor the switch that turns it off is known. To further our understanding of KD pathogenesis and the role of regulatory T-cells in modulating the inflammatory response, we studied circulating effector memory T-cells (CCR7- and IL-15+ T(em)) and central memory T-cells (CCR7+ and IL-15+ T(cm)) in six KD subjects. In two of the subjects, we cloned the remaining T-cell population by limiting dilution. TaqMan analysis of T(em) studied in two KD subjects suggested that T(em) are pro-inflammatory CD4+T-helper 1 cells and CD8+ cytotoxic T-cells. Following memory T-cells over time, we defined that circulating T(em) and T(cm) are detectable during the acute phase in some KD subjects before treatment with intravenous immunoglobulin. Both T(em) and T(cm) expand rapidly within 2 weeks of treatment. The circulating T(em) pool contracts, while T(cm) further proliferate in the convalescent phase. Following depletion of memory T-cells, numerous T-cell clones were derived from two acute KD subjects. The large majority of these T-cells displayed the functional phenotype of peripherally induced regulatory T-cells (T(reg)). These findings provide insight into the nature and kinetics of the adaptive immune response in KD.
川崎病（KD）是一种儿科自限性血管炎，其特征为动脉壁和心肌的免疫介导性破坏。引发炎症的触发因素和使其停止的开关均未知。为了进一步了解 KD 的发病机制以及调节性 T 细胞在调节炎症反应中的作用，我们研究了 6 例 KD 患者的循环效应记忆 T 细胞（CCR7-和 IL-15+ T（em））和中央记忆 T 细胞（CCR7+和 IL-15+ T（cm））。在其中 2 例患者中，我们通过限制稀释克隆了剩余的 T 细胞群体。对 2 例 KD 患者中研究的 T（em）进行 TaqMan 分析表明，T（em）是促炎的 CD4+辅助性 1 型 T 细胞和 CD8+细胞毒性 T 细胞。随着时间的推移研究记忆 T 细胞，我们定义在静脉注射免疫球蛋白治疗之前，一些 KD 患者的急性期可检测到循环 T（em）和 T（cm）。T（em）和 T（cm）在治疗后 2 周内迅速扩张。循环 T（em）池收缩，而 T（cm）在恢复期进一步增殖。在耗尽记忆 T 细胞后，从 2 例急性 KD 患者中衍生出了大量 T 细胞克隆。这些 T 细胞的绝大多数表现出外周诱导的调节性 T 细胞（T（reg））的功能表型。这些发现为 KD 中的适应性免疫反应的性质和动力学提供了深入了解。
