Abstract:
First described in 2020, multi-system inflammatory syndrome in children (MIS-C) is an, initially life-threatening, disease characterised by severe inflammation and following exposure to SARS-CoV-2. The immunopathology of MIS-C involves a hyperinflammation characterised by a cytokine storm and activation of both the innate and adaptive immune system, eventually leading to multi-organ failure. Several etiological theories are described in literature. Firstly, it is suggested that the gut plays an important role in the translocation of microbial products to the systemic circulation. Additionally, the production of autoantibodies that develop after the initial infection with SARS-CoV-2 might lead to many of its broad clinical symptoms. Finally, the superantigen theory where non-specific binding of the SARS-CoV-2 spike glycoprotein to the T-cell receptor leads to a subsequent activation of T cells, generating a powerful immune response. Despite the sudden outbreak of MIS-C and alarming messages, as of 2024, cases have declined drastically and subsequently show a less severe clinical spectrum. However, subacute cases not meeting current diagnostic criteria might be overlooked even though they represent a valuable research population. In the future, research should focus on adjusting these criteria to better understand the broad pathophysiology of MIS-C, aiding early detection, therapy, and prediction.
摘要:
首次描述于2020年,儿童多系统炎症综合征(MIS-C)是一种,最初危及生命,以严重炎症和暴露于SARS-CoV-2后为特征的疾病。MIS-C的免疫病理学涉及以细胞因子风暴和先天和适应性免疫系统的激活为特征的过度炎症。最终导致多器官衰竭。文献中描述了几种病因学理论。首先,有人认为,肠道在微生物产物向体循环的转运中起着重要作用。此外,SARS-CoV-2初次感染后产生的自身抗体可能导致其许多广泛的临床症状。最后,超抗原理论,其中SARS-CoV-2刺突糖蛋白与T细胞受体的非特异性结合导致随后的T细胞激活,产生强大的免疫反应。尽管MIS-C突然爆发和令人震惊的消息,截至2024年,病例急剧下降,随后显示出不那么严重的临床谱.然而,不符合当前诊断标准的亚急性病例可能会被忽视,尽管它们代表了有价值的研究人群.在未来,研究应侧重于调整这些标准,以更好地理解MIS-C的广泛病理生理学,帮助早期发现,治疗,和预测。