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Pathogenesis, immunology, and immune-targeted management of the multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multisystem syndrome (PIMS): EAACI Position Paper
W. Feleszko, M. Okarska-Napierała, EP. Buddingh, M. Bloomfield, A. Sediva, C. Bautista-Rodriguez, HA. Brough, PA. Eigenmann, T. Eiwegger, A. Eljaszewicz, S. Eyerich, C. Gomez-Casado, A. Fraisse, J. Janda, R. Jiménez-Saiz, T. Kallinich, IK. Krohn,...
Language English Country England, Great Britain
Document type Journal Article
PubMed
36705045
DOI
10.1111/pai.13900
Knihovny.cz E-resources
- MeSH
- COVID-19 * MeSH
- Child MeSH
- Humans MeSH
- SARS-CoV-2 MeSH
- Systemic Inflammatory Response Syndrome diagnosis therapy MeSH
- COVID-19 Vaccines MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Publication type
- Journal Article MeSH
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.
Center for Allergy and Environment Technical University and Helmholtz Center Munich Munich Germany
Christine Kühne Center for Allergy Research and Education Davos Switzerland
Department of Dermatology Vrije Universiteit Brussel Brussels Belgium
Department of Immunology and Oncology Centro Nacional de Biotecnología Madrid Spain
Department of Immunology University of Toronto Toronto Ontario Canada
Department of Neonatology Erasmus MC Sophia Rotterdam The Netherlands
Department of Paediatric Medicine Franciscus Gasthuis and Vlietland Rotterdam The Netherlands
Department of Pediatric and Adolescent Medicine University Hospital St Pölten St Pölten Austria
Department of Pediatric Pneumology and Allergy The Medical University of Warsaw Warsaw Poland
Department of Pediatrics with Clinical Assessment Unit Medical University of Warsaw Warsaw Poland
Department of Women Children Teenagers University Hospital of Geneva Geneva Switzerland
Faculty of Experimental Sciences Universidad Francisco de Vitoria Madrid Spain
Faculty of Science Charles University Prague Czech Republic
Karl Landsteiner University of Health Sciences Krems Austria
National Heart and Lung Institute Imperial College London London UK
Pediatric Cardiology Services Royal Brompton Hospital London UK
SKIN Research Group Vrije Univeristeit Brussel Brussels Belgium
Swiss Institute of Allergy and Asthma Research University of Zurich Davos Switzerland
References provided by Crossref.org
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- $a 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.
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