Gkoutzourelas Athanasios, Bogdanos Dimitrios P, Sakkas Lazaros I
Department of Rheumatology and Clinical Immunology, Faculty of medicine, School of Health Sciences, University of Thessaly, Larissa.
Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece.
Mediterr J Rheumatol. 2020 Sep 21;31(Suppl 2):268-274. doi: 10.31138/mjr.31.3.268. eCollection 2020 Sep.
The recent passing away of Dr. Tomisaku Kawasaki, who first described what is now known as Kawasaki Disease (KD), and recent reports of a multisystem inflammatory disease in children associated with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (MIS-C), makes a review on KD and MIS-C timely. Kawasaki Disease is a systemic vasculitis with predilection for coronary arteries occurring mostly in early childhood. The main features are high fever, extensive skin rash, cheilitis with red, cracking, bleeding lips and strawberry tongue, conjunctivitis, erythema and induration of hands and feet, subsiding with periungual peeling, cervical lymphadenopathy, and coronary artery dilation/aneurysms. Treatment consists of intravenous (IV) immunoglobulin (Ig) plus acetylsalicylic acid. MIS-C is considered a cytokine storm with high fever, inflammation, multi-organ dysfunction, that shares features with KD, toxic shock, and macrophage activation syndrome. Many children require admission to paediatric intensive care units for circulatory support. Bacterial sepsis, staphylococcal toxic shock syndrome, and enterovirus-causing myocarditis should be excluded. Treatment is not standardized and includes IVIg, IV methylprednisolone and IL-6 and IL-1 inhibitors.
最早描述如今被称为川崎病(KD)的富山和夫博士最近离世,以及近期有关儿童多系统炎症性疾病与严重急性呼吸综合征冠状病毒2(SARS-CoV-2)相关(MIS-C)的报道,使得对川崎病和儿童多系统炎症综合征进行综述变得及时。川崎病是一种主要发生在幼儿期的全身性血管炎,易累及冠状动脉。主要特征包括高热、广泛皮疹、嘴唇发红、干裂、出血以及草莓舌的唇炎、结膜炎、手脚红斑和硬结,并伴有指(趾)甲周脱皮、颈部淋巴结病,以及冠状动脉扩张/动脉瘤。治疗包括静脉注射免疫球蛋白(Ig)加乙酰水杨酸。儿童多系统炎症综合征被认为是一种伴有高热、炎症、多器官功能障碍的细胞因子风暴,与川崎病、中毒性休克和巨噬细胞活化综合征有共同特征。许多儿童需要入住儿科重症监护病房进行循环支持。应排除细菌性败血症、葡萄球菌中毒性休克综合征和肠道病毒引起的心肌炎。治疗尚无标准化方案,包括静脉注射免疫球蛋白、静脉注射甲泼尼龙以及白细胞介素-6和白细胞介素-1抑制剂。