Pathogenesis, immunology, and immune-targeted management of the multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multisystem syndrome (PIMS) EAACI Position Paper

Feleszko, W.; Okarska-Napierala, M.; Buddingh, E.P.; Bloomfield, M.; Sediva, A.; Bautista-Rodriguez, C.; ... ; Immunology Sect Working (2023)

Article / Letter to editor

Multisystem inflammatory syndrome in children (MIS-C) is a rare, but severe complication of coronavirus disease 2019 (COVID-19). It develops approximately 4 weeks after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and involves hyperinflammation with multisystem injury, commonly progressing to shock. The exact pathomechanism of MIS-C is not known, but immunological dysregulation leading to cytokine storm plays a central role. In response to the emergence of MIS-C, the European Academy of Allergy and Clinical Immunology (EAACI) established a task force (TF) within the Immunology Section in May 2021. With the use of an online Delphi process, TF formulated clinical statements regarding immunological background of MIS-C, diagnosis, treatment, follow-up, and the role of COVID-19 vaccinations. MIS-C case definition is broad, and diagnosis is made based on clinical presentation. The immunological mechanism leading to MIS-C is unclear and depends on activating multiple pathways leading to hyperinflammation. Current management of MIS-C relies on supportive care in combination with immunosuppressive and/or immunomodulatory agents. The most frequently used agents are systemic steroids and intravenous immunoglobulin. Despite good overall short-term outcome, MIS-C patients should be followed-up at regular intervals after discharge, focusing on cardiac disease, organ damage, and inflammatory activity. COVID-19 vaccination is a safe and effective measure to prevent MIS-C. In anticipation of further research, we propose a convenient and clinically practical algorithm for managing MIS-C developed by the Immunology Section of the EAACI.

• children
• clinical algorithm
• clinical guidance
• Delphi
• hyperinflammation
• intravenous immunoglobulin
• management
• MIS-C
• SARS-CoV-2
• steroids

• All authors: Feleszko, W.; Okarska-Napierala, M.; Buddingh, E.P.; Bloomfield, M.; Sediva, A.; Bautista-Rodriguez, C.; Brough, H.A.; Eigenmann, P.A.; Eiwegger, T.; Eljaszewicz, A.; Eyerich, S.; Gomez-Casado, C.; Fraisse, A.; Janda, J.; Jiménez-Saiz, R.; Kallinich, T.; Krohn, I.K.; Mortz, C.G.; Riggioni, C.; Sastre, J.; Sokolowska, M.; Strzelczyk, Z.; Untersmayr, E.; Tramper-Stranders, G.; Immunology Sect Working
• Date: 2023-01-01
• Journal: Pediatric Allergy and Immunology
• Volume: 34
• Issue: 1
• DOI: doi:10.1111/pai.13900
• Link: https://onlinelibrary.wiley.com/doi/10.1111/pai.13900