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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,...

. 2023 ; 34 (1) : e13900. [pub] -

Language English Country England, Great Britain

Document type Journal Article

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.

Allergy Immunology and Rheumatology Division Department of Paediatrics Yong Loo Lin School of Medicine National University of Singapore Singapore Singapore

Center for Allergy and Environment Technical University and Helmholtz Center Munich Munich Germany

Children's Allergy Service Evelina Children's Hospital Guy's and St Thomas' Hospital NHS Foundation Trust London UK

Christine Kühne Center for Allergy Research and Education Davos Switzerland

Department of Dermatology and Allergy Center Odense Research Center for Anaphylaxis Odense University Hospital Odense Denmark

Department of Dermatology Medical Faculty University Hospital Düsseldorf Heinrich Heine University Düsseldorf Düsseldorf Germany

Department of Dermatology Vrije Universiteit Brussel Brussels Belgium

Department of Immunology 2nd Faculty of Medicine Motol University Hospital Charles University Prague Czech Republic

Department of Immunology and Oncology Centro Nacional de Biotecnología Madrid Spain

Department of Immunology Instituto de Investigación Sanitaria Hospital Universitario de La Princesa Madrid Spain

Department of Immunology University of Toronto Toronto Ontario Canada

Department of Medicine McMaster Immunology Research Centre McMaster University Hamilton 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 1st Faculty of Medicine Thomayer University Hospital Charles University Prague Czech Republic

Department of Pediatrics Willem Alexander Children's Hospital Leiden University Medical Centre Leiden The Netherlands

Department of Pediatrics with Clinical Assessment Unit Medical University of Warsaw Warsaw Poland

Department of Regenerative Medicine and Immune Regulation Medical University of Bialystok Bialystok 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

Fundacion Jimenez Diaz and CIBER de Enfermedades Respiratorias Instituto de Salud Carlos 3 Madrid Spain

Institute of Pathophysiology and Allergy Research Center of Pathophysiology Infectiology and Immunology Medical University of Vienna Vienna Austria

Karl Landsteiner University of Health Sciences Krems Austria

National Heart and Lung Institute Imperial College London London UK

Paediatric Allergy Group Department of Women and Children's Health School of Life Course Sciences St Thomas' Hospital King's College London London UK

Paediatric Allergy Group Peter Gorer Department of Immunobiology School of Immunology and Microbial Sciences Guys' Hospital King's College London London UK

Pediatric Cardiology Services Royal Brompton Hospital London UK

Pediatric Pneumology Immunology and Critical Care Medicine Charité Universitätsmedizin Berlin and Deutsches Rheuma Forschungszentrum an Institute of the Leibniz Association Berlin Germany

SKIN Research Group Vrije Univeristeit Brussel Brussels Belgium

Swiss Institute of Allergy and Asthma Research University of Zurich Davos Switzerland

Translational Medicine Program Research Institute The Hospital for Sick Children Toronto Ontario Canada

References provided by Crossref.org

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