Allergy-Immunology and Pediatric Rheumatology Division, Department of Pediatrics, Children's Hospital of Georgia, Augusta University Medical Center, 1120 15th Street, BG 102, Augusta, GA, 30912, USA.
Curr Allergy Asthma Rep. 2022 May;22(5):53-60. doi: 10.1007/s11882-022-01031-4. Epub 2022 Mar 22.
PURPOSE OF REVIEW: The novel coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and has developed into a pandemic. A unique challenge of this pandemic has been the emergence of multisystem inflammatory syndrome in children (MIS-C), a rare post-infectious hyperinflammatory disorder associated with SARS-CoV-2. This syndrome is characterized by overwhelming systemic inflammation, fever, hypotension, and cardiac dysfunction. This disorder may also have features overlapping with Kawasaki disease (KD), macrophage activation syndrome (MAS), and toxic shock syndrome (TSS). The goal of this review is to outline the presenting features, presumed immunopathogenesis, management, and outcomes of patients with MIS-C. RECENT FINDINGS: Patients with MIS-C present with characteristics that fall within a wide clinical spectrum. Main features include fever, gastrointestinal symptoms such as abdominal pain and diarrhea, and cardiac complications such as myocarditis and coronary artery aneurysms, although various other features have been reported. Younger children may present with features of Kawasaki-like disease, and older children are often admitted to the intensive care unit with cardiogenic shock. Current treatment guidelines recommend intravenous immunoglobulins (IVIG) and glucocorticoids, with utilization of biologics in refractory cases. Fortunately, the majority of patients recover, with resolution of the systemic inflammation and cardiac abnormalities. Mortality from MIS-C is rare. This review provides an overview of the presenting features, proposed pathogenesis, suggested therapies, and outcomes of MIS-C. Clinicians must have a high clinical suspicion for this disorder in children who have had recent COVID-19 infection or exposure and present with a significant inflammatory response. Understanding of this disorder continues to evolve, and prompt diagnosis and treatment allow for the best possible outcome for patients with MIS-C.
目的综述:新型冠状病毒病(COVID-19)由严重急性呼吸综合征冠状病毒 2 型(SARS-CoV-2)引起,已发展为大流行。该大流行的一个独特挑战是儿童多系统炎症综合征(MIS-C)的出现,这是一种与 SARS-CoV-2 相关的罕见感染后高炎症性疾病。这种综合征的特点是全身性炎症、发热、低血压和心功能障碍。该疾病还可能与川崎病(KD)、巨噬细胞活化综合征(MAS)和中毒性休克综合征(TSS)存在重叠特征。本文旨在概述 MIS-C 患者的临床表现、推测的发病机制、治疗和预后。
最新发现:MIS-C 患者的表现特征呈广泛的临床谱。主要特征包括发热、胃肠道症状(如腹痛和腹泻)和心脏并发症(如心肌炎和冠状动脉瘤),尽管也有报道其他各种特征。年龄较小的患儿可能表现为类似于川崎病的特征,年龄较大的患儿常因心源性休克而收入重症监护病房。目前的治疗指南建议使用静脉注射免疫球蛋白(IVIG)和糖皮质激素,在难治性病例中使用生物制剂。幸运的是,大多数患者会康复,全身炎症和心脏异常得到缓解。MIS-C 的死亡率罕见。本文概述了 MIS-C 的临床表现、提出的发病机制、建议的治疗方法和预后。临床医生必须对近期感染 COVID-19 或接触过 COVID-19 且表现出明显炎症反应的患儿高度怀疑这种疾病。对这种疾病的认识在不断发展,及时诊断和治疗可使 MIS-C 患者获得最佳预后。
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