Zhu Frank, Ang Jocelyn Y
Department of Pediatrics, Division of Pediatric Infectious Diseases, Medical College of Wisconsin, Suite 450C, Pediatric Infectious Diseases, 999 North 92nd Street, Wauwatosa, Milwaukee, WI 53226 USA.
Division of Pediatric Infectious Diseases, Children's Hospital of Michigan, Detroit, MI USA.
Curr Infect Dis Rep. 2021;23(3):3. doi: 10.1007/s11908-021-00746-1. Epub 2021 Feb 6.
Provide an updated review of the clinical management and diagnosis of Kawasaki disease with inclusion of potential diagnostic difficulties with multisystem inflammatory syndrome in children (MIS-C) given the ongoing COVID-19 pandemic.
Adjunctive corticosteroid therapy has been shown to reduce the rate of coronary artery dilation in children at high risk for IVIG resistance in multiple Japanese clinical studies (most notably RAISE study group). Additional adjunctive therapies (etanercept, infliximab, cyclosporin) may also provide limited benefit, but data is limited to single studies and subgroups of patients with cardiac abnormalities. The efficacy of other agents (atorvastatin, doxycycline) is currently being investigated. MIS-C is a clinically distinct entity from KD with broad clinical manifestations and multiorgan involvement (cardiac, GI, hematologic, dermatologic, respiratory, renal). MIS-C with Kawasaki manifestations is more commonly seen in children < 5 years of age.
The 2017 American Heart Association (AHA) treatment guidelines have included changes in aspirin dosing (including both 80-100 mg/kg/day and 30-50 mg/kg/day treatment options), consideration of the use of adjuvant corticosteroid therapy in patients at high risk of IVIG resistance, and the change in steroid regimen for refractory KD to include both pulse-dose IVMP and longer course of prednisolone with an oral taper. A significant proportion of children diagnosed with MIS-C, a post-infectious syndrome of SARS-CoV-2 infection, meet criteria for Kawasaki disease. Further investigation is warranted to further delineate these conditions and optimize treatment of these conditions given the ongoing COVID-19 pandemic.
鉴于新冠疫情仍在持续，对川崎病的临床管理和诊断进行更新综述，包括儿童多系统炎症综合征（MIS-C）潜在的诊断困难。
在多项日本临床研究（最著名的是RAISE研究组）中，辅助性皮质类固醇疗法已被证明可降低静脉注射免疫球蛋白（IVIG）抵抗高危儿童的冠状动脉扩张率。其他辅助疗法（依那西普、英夫利昔单抗、环孢素）可能也有有限的益处，但数据仅限于单项研究和有心脏异常的患者亚组。目前正在研究其他药物（阿托伐他汀、强力霉素）的疗效。MIS-C是一种与川崎病临床特征不同的疾病，临床表现广泛，累及多个器官（心脏、胃肠道、血液、皮肤、呼吸、肾脏）。有川崎病表现的MIS-C在5岁以下儿童中更常见。
2017年美国心脏协会（AHA）治疗指南包括阿司匹林剂量的变化（包括80-100mg/kg/天和30-50mg/kg/天两种治疗方案）、考虑对IVIG抵抗高危患者使用辅助性皮质类固醇疗法，以及难治性川崎病类固醇治疗方案的改变，包括静脉注射甲泼尼龙冲击剂量和更长疗程的泼尼松龙口服逐渐减量。很大一部分被诊断为MIS-C（一种新冠病毒感染后的综合征）的儿童符合川崎病的标准。鉴于新冠疫情仍在持续，有必要进一步研究以进一步明确这些疾病并优化其治疗。
