Abu Hammour Khawla, Abu Farha Rana, Manaseer Qusai, Dawoud Tasnim, Abu Hammour Walid
Department of Biopharmaceutics and Clinical Pharmacy, The University of Jordan, Amman, Jordan.
Department of Clinical Pharmacy and Therapeutics, Applied Science Private University, Amman, Jordan.
Arch Rheumatol. 2021 Dec 24;37(2):230-244. doi: 10.46497/ArchRheumatol.2022.9086. eCollection 2022 Jun.
In this systematic review, we aimed to evaluate the clinical features, therapeutic options, and outcomes of children with multisystem inflammatory syndrome in children (MIS-C) and to investigate whether MIS-C is a new variant of Kawasaki disease.
Adhering to PRISMA principles, we searched for eligible studies between December 2019 and June 2020 through the following databases: PubMed, ISI Web of Science, SCOPUS, and Science Direct. Studies including original data of patients aged <21 years with MIS-C and descriptions of clinical signs, laboratory or radiological investigations were selected.
A total of 84 studies were identified, for which 48 were eligible for full screening and only 13 studies (n=657) met our inclusion criteria. More than 70% of patients with MIS-C tested positive for severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2). The most common symptoms were gastrointestinal (80 to 100%) and most patients presented with fever for >4 days. Mucocutaneous manifestations are similar to Kawasaki disease presented in up to 64% in some studies. Almost all patients had significant elevations in inflammatory markers, and up to 50 to 100% had elevated troponin suggesting myocardial damage. Intravenous immunoglobulin (IVIG) was administered to 60% of patients in 12 studies and 80 to 100% in five studies. Steroids were administered to 10 to 95% of patients. The overall mortality rate was 0.9%.
The temporal association between novel coronavirus disease 2019 (COVID-19) onset and Kawasaki-like disease and MIS-C suggests a causal link. Both syndromes have similar cascades of symptoms and hyperinflammation, which likely explain their response to the same immunomodulatory agents. However, it is unclear yet why some children appear more susceptible to develop MIS-C.
在本系统评价中，我们旨在评估儿童多系统炎症综合征（MIS-C）患儿的临床特征、治疗选择及预后，并调查MIS-C是否为川崎病的一种新变体。
遵循PRISMA原则，我们于2019年12月至2020年6月期间通过以下数据库检索符合条件的研究：PubMed、ISI科学网、SCOPUS和Science Direct。纳入的研究需包含年龄小于21岁的MIS-C患者的原始数据以及临床体征、实验室或影像学检查的描述。
共识别出84项研究，其中48项符合全面筛选条件，仅有13项研究（n = 657）符合我们的纳入标准。超过70%的MIS-C患者严重急性呼吸综合征冠状病毒2（SARS-CoV-2）检测呈阳性。最常见的症状为胃肠道症状（80%至100%），大多数患者发热超过4天。在一些研究中，黏膜皮肤表现与川崎病相似，比例高达64%。几乎所有患者的炎症标志物均显著升高，高达50%至100%的患者肌钙蛋白升高提示心肌损伤。在12项研究中，60%的患者接受了静脉注射免疫球蛋白（IVIG）治疗，在5项研究中这一比例为80%至100%。10%至95%的患者接受了类固醇治疗。总体死亡率为0.9%。
2019年新型冠状病毒病（COVID-19）发病与川崎样疾病及MIS-C之间的时间关联提示存在因果关系。这两种综合征具有相似的症状级联反应和过度炎症反应，这可能解释了它们对相同免疫调节药物的反应。然而，目前尚不清楚为何有些儿童似乎更容易发生MIS-C。
