Circulating immunocompetent cell profiles during oral cyclosporin therapy for immunoglobulin-resistant Kawasaki disease
Circulating immunocompetent cell profiles during oral cyclosporin therapy for immunoglobulin-resistant Kawasaki disease
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口服环孢素治疗免疫球蛋白耐药川崎病期间的循环免疫活性细胞谱
DOI：
10.1007/s12519-021-00468-3
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发表时间：
2021
期刊：
影响因子：
8.7
通讯作者：
Hasegawa S
中科院分区：
文献类型：
--
作者：
Okada S;Ohnishi Y;Furuta T;Suzuki Y;Kawakami-Miyake A;Matsuguma C;Waniishi T;Yasudo H;Hasegawa S
虽然川崎（KD）的标准治疗是静脉注射免疫球蛋白（IVIG）联合口服阿司匹林，但18%的第一次IVIG是难治性的[1]。最近，已报道了免疫调节药物（如泼尼松龙、英夫利昔单抗（IFX）和环孢素（CsA））治疗IVIG耐药KD的疗效[2-5]。然而，IVIG耐药KD的最佳免疫调节治疗尚未建立。在本研究中，我们评估了口服CsA（n= 6）与IFX（n= 51）（对照组，具有不同的特异性治疗靶点）对IVIG耐药KD的免疫调节作用（补充图1）。所有患者均在治疗前给予IVIG 2 g/kg/剂2个周期。根据先前的研究[6]，通过流式细胞术评估外周血单核细胞（PBMC）的动力学。冠状动脉病变定义为发病后1个月通过二维超声心动图测量的冠状动脉直径的Z评分≥+ 2.5 [1，7]。第9天（9-21）开始CsA给药，剂量为4 mg/kg/天，持续11天（7-14）（补充表1）。所有患者CsA治疗后均完全退热。在对照组中，患者在第8天（4-15）接受5 mg/kg剂量的IFX。两组之间无显著差异。
Although the standard treatment for Kawasaki disease (KD) is intravenous immunoglobulin (IVIG) combined with oral aspirin, 18% of 1st IVIG is refractory [1]. Recently, the efficacy of immunomodulatory drugs for IVIG-resistant KD, such as prednisolone, infliximab (IFX), and cyclosporine (CsA), has been reported [2–5]. However, optimal immunomodulatory therapy for IVIG-resistant KD has not yet been established.In this study, we evaluated the immunomodulatory effects of oral CsA (n= 6) compared with IFX (n= 51)(control group, which has different and specific therapeutic targets) for IVIG-resistant KD (Supplementary Fig. 1). All patients were administered two cycles of IVIG (2 g/kg/dose) before treatment. The kinetics of peripheral blood mononuclear cells (PBMCs) were assessed by flow cytometry according to a previous study [6]. Coronary artery lesions were defined as a Z score of coronary diameters≥+ 2.5, measured by two-dimensional echocardiography 1 month after onset [1, 7]. CsA administration was started on day 9 (9–21) at a dose of 4 mg/kg/day and continued for 11 (7–14) days (Supplementary Table 1). All patients showed complete defervescence after CsA therapy. In the control group, patients received IFX at a dose of 5 mg/kg on day 8 (4–15). There were no significant differences in the