Renson Thomas, Hamiwka Lorraine, Benseler Susanne
Division of Rheumatology, Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.
Division of Nephrology, Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.
Front Pediatr. 2022 Aug 10;10:931179. doi: 10.3389/fped.2022.931179. eCollection 2022.
Central nervous system (CNS) involvement in monogenic autoinflammatory disorders (AID) is increasingly recognized and can be life threatening. Therefore, a low threshold to consider CNS disease should be maintained in patients with systemic inflammation. Hyperinflammation is also a key feature of severe acute COVID-19 and post COVID-19 entities such as multisystem inflammatory syndrome in children. Like AID, COVID-19 patients can present with severe CNS involvement. The impact of COVID-19 on AID and CNS involvement in particular is still obscure, nevertheless dreaded. In the current review, we synthesize the spectrum of CNS manifestations in monogenic AID. We explore common pathophysiological and clinical features of AID and COVID-19. Moreover, we assess the impact of immune dysregulation associated with SARS-CoV-2 infections and post COVID-19 hyperinflammation in AID. The striking commonalities found between both disease entities warrant caution in the management of AID patients during the current pandemic.
中枢神经系统(CNS)受累于单基因自身炎症性疾病(AID)的情况日益受到认可,且可能危及生命。因此,对于患有全身性炎症的患者,应保持较低的阈值来考虑中枢神经系统疾病。炎症过度也是重症急性 COVID-19 以及 COVID-19 后疾病(如儿童多系统炎症综合征)的关键特征。与 AID 一样,COVID-19 患者也可能出现严重的中枢神经系统受累情况。COVID-19 对 AID 尤其是中枢神经系统受累的影响仍不明确,但令人担忧。在当前的综述中,我们综合了单基因 AID 中枢神经系统表现的范围。我们探讨了 AID 和 COVID-19 常见的病理生理和临床特征。此外,我们评估了与 SARS-CoV-2 感染相关的免疫失调以及 COVID-19 后炎症过度对 AID 的影响。在当前大流行期间,这两种疾病实体之间显著的共性值得在 AID 患者的管理中谨慎对待。