Felsenstein Susanna, Willis Emily, Lythgoe Hannah, McCann Liza, Cleary Andrew, Mahmood Kamran, Porter David, Jones Jessica, McDonagh Janet, Chieng Alice, Varnier Giulia, Hughes Stephen, Boullier Mary, Ryan Fiona, Awogbemi Olumoyin, Soda Giridhar, Duong Phuoc, Pain Claire, Riley Phil, Hedrich Christian M
Department of Infectious Diseases and Immunology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool L14 5AB, UK.
Department of Rheumatology, Royal Manchester Children's Hospital, Manchester M13 9WL, UK.
J Clin Med. 2020 Oct 14;9(10):3293. doi: 10.3390/jcm9103293.
The novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) is the pathogen responsible for Coronavirus Disease 2019 (COVID-19). Whilst most children and young people develop mild symptoms, recent reports suggest a novel paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS). Case definition and classification are preliminary, treatment is empiric and disease-associated outcomes are unclear. Here, we report 29 patients with PIMS-TS who were diagnosed, admitted and treated in the English North West between March and June 2020. Consistent with patterns observed internationally, cases peaked approximately 4 weeks after the initial surge of COVID-19-like symptoms in the UK population. Clinical symptoms included fever (100%), skin rashes (72%), cardiovascular involvement (86%), conjunctivitis (62%) and respiratory involvement (21%). Some patients had clinical features partially resembling Kawasaki disease (KD), toxic shock syndrome and cytokine storm syndrome. Male gender (69%), black, Asian and other minority ethnicities (BAME, 59%) were over-represented. Immune modulating treatment was used in all, including intravenous immunoglobulin (IVIG), corticosteroids and cytokine blockers. Notably, 32% of patients treated with IVIG alone went into remission. The rest required additional treatment, usually corticosteroids, with the exception of two patients who were treated with TNF inhibition and IL-1 blockade, respectively. Another patient received IL-1 inhibition as primary therapy, with associated rapid and sustained remission. Randomized and prospective studies are needed to investigate efficacy and safety of treatment, especially as resources of IVIG may be depleted secondary to high demand during future waves of COVID-19.
新型严重急性呼吸综合征冠状病毒2(SARS-CoV-2)是2019冠状病毒病(COVID-19)的病原体。虽然大多数儿童和年轻人症状较轻,但最近的报告显示,有一种与SARS-CoV-2在时间上相关的新型儿童炎症性多系统综合征(PIMS-TS)。病例定义和分类尚属初步,治疗是经验性的,与疾病相关的结果尚不清楚。在此,我们报告了29例2020年3月至6月在英国西北部被诊断、收治和治疗的PIMS-TS患者。与国际上观察到的模式一致,病例在英国人群中最初出现类似COVID-19症状激增约4周后达到峰值。临床症状包括发热(100%)、皮疹(72%)、心血管受累(86%)、结膜炎(62%)和呼吸道受累(21%)。一些患者具有部分类似于川崎病(KD)、中毒性休克综合征和细胞因子风暴综合征的临床特征。男性(69%)、黑种人、亚洲人和其他少数族裔(BAME,59%)的比例过高。所有患者均使用了免疫调节治疗,包括静脉注射免疫球蛋白(IVIG)、皮质类固醇和细胞因子阻滞剂。值得注意的是,仅接受IVIG治疗的患者中有32%病情缓解。其余患者需要额外治疗,通常是皮质类固醇,只有两名患者分别接受了肿瘤坏死因子抑制和白细胞介素-1阻断治疗。另一名患者接受白细胞介素-1抑制作为主要治疗,病情迅速且持续缓解。需要进行随机和前瞻性研究来调查治疗的疗效和安全性,特别是在未来COVID-19疫情期间,由于需求高涨,IVIG资源可能会枯竭。