Coronary artery lesions and the increasing incidence of Kawasaki disease resistant to initial immunoglobulin
Coronary artery lesions and the increasing incidence of Kawasaki disease resistant to initial immunoglobulin
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DOI：
10.1016/j.ijcard.2016.03.017
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发表时间：
2016-07-01
影响因子：
3.5
通讯作者：
Ohga, Shouichi
中科院分区：
文献类型：
--
作者：
Kibata, Tetsuhiro;Suzuki, Yasuo;Ohga, Shouichi
背景：川崎病（KD）是一种累及冠状动脉的儿童全身性血管炎。对于心脏后遗症风险较高的疑难病例的治疗仍存在争议。方法：利用覆盖该县的所有 14 家医院的医疗记录，对 2003 年至 2014 年日本山口县诊断出的川崎病患者的临床结果进行分析。该研究纳入了 1487 名患者（男性：女性，873：614；诊断时中位年龄，24 个月）。 结果：在这十年中，初始静脉注射免疫球蛋白 (IVIG) 耐药的患者比例从 7% 增加到 23%，尽管没有患者死亡。 KD 发病后 1 个月内，24 名患者出现冠状动脉病变 (CAL)。病程期间接受皮质类固醇治疗的患者CAL的发生率(10/37; 27.0%)高于未接受皮质类固醇治疗的患者(14/1450; 0.97%, p = 2.0 x 10(-35))。对初始 IVIG 加皮质类固醇有反应的 9 名患者没有出现 CAL。相反，接受替代皮质类固醇治疗的 IVIG 耐药患者比未接受替代皮质类固醇治疗的患者更容易发生 CAL (10/28; 35.7% vs. 5/194; 2.6%, p = 8.9 x 10(-10))。多变量分析表明，皮质类固醇治疗 (p < 0.0001)、高胆红素血症 (p = 0.0010) 和治疗前较长天数 (p = 0.0005) 是与发病后一个月以上 CAL 相关的危险因素。如果病例仅限于初始 IVIG 无反应者和无皮质类固醇 IVIG 反应者，则皮质类固醇使用的优势比从 18.3 增加至 43.5。 结论：IVIG 失败最近有所增加。长期使用皮质类固醇的顽固病例中 CAL 的发生率会增加。皮质类固醇可能不能作为 IVIG 失败的替代选择来降低心脏后遗症的风险。 (C) 2016 Elsevier Ireland Ltd. 保留所有权利。
Backgrounds: Kawasaki disease (KD) is a systemic vasculitis of childhood involving coronary arteries. Treatment for intractable cases at a higher risk of cardiac sequelae remains controversial.Methods: Clinical outcomes of KD patients diagnosed in Yamaguchi prefecture, Japan between 2003 and 2014 were analyzed using the medical records from all 14 hospitals covering the prefecture. The study included 1487 patients (male: female, 873: 614; median age at diagnosis, 24 months).Results: The proportion of initial intravenous immunoglobulin (IVIG)-resistant patients increased from 7% to 23% during this decade, although no patients died. Twenty-four patients developed coronary artery lesions (CALs) over one month after the KD onset. The incidence of CAL in patients who received corticosteroid during the disease course (10/37; 27.0%) was higher than that in those who did not (14/1450; 0.97%, p = 2.0 x 10(-35)). Nine patients who responded to initial IVIG plus corticosteroids had no CAL. Conversely, IVIG-resistant patients with alternate corticosteroid therapy more frequently developed CAL than those without it (10/28; 35.7% vs. 5/194; 2.6%, p = 8.9 x 10(-10)). Multivariate analyses indicated corticosteroid therapy (p < 0.0001), hyperbilirubinemia (p = 0.0010), and a longer number of days before treatment (p = 0.0005) as risk factors associated with CAL over a month after onset. The odds ratio of corticosteroid use increased from 18.3 to 43.5 if the cases were limited to initial IVIG non-responders and corticosteroid free-IVIG responders.Conclusions: IVIG-failure has recently increased. The incidence of CAL increased in intractable cases with prolonged corticosteroid use. Corticosteroid may not be alternate choice for IVIG-failure to reduce the risk of cardiac sequelae. (C) 2016 Elsevier Ireland Ltd. All rights reserved.