tetano
Editor, Senior Moderator
Pediatr Allergy Immunol
. 2023 Jan;34(1):e13900.
doi: 10.1111/pai.13900.
Pathogenesis, immunology, and immune-targeted management of the multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multisystem syndrome (PIMS): EAACI Position Paper
Wojciech Feleszko[SUP] 1 [/SUP], Magdalena Okarska-Napierała[SUP] 2 [/SUP], Emilie Pauline Buddingh[SUP] 3 [/SUP], Marketa Bloomfield[SUP] 4 5 [/SUP], Anna Sediva[SUP] 4 [/SUP], Carles Bautista-Rodriguez[SUP] 6 7 [/SUP], Helen A Brough[SUP] 8 9 10 [/SUP], Philippe A Eigenmann[SUP] 11 [/SUP], Thomas Eiwegger[SUP] 12 13 14 15 [/SUP], Andrzej Eljaszewicz[SUP] 16 [/SUP], Stefanie Eyerich[SUP] 17 [/SUP], Cristina Gomez-Casado[SUP] 18 [/SUP], Alain Fraisse[SUP] 6 7 [/SUP], Jozef Janda[SUP] 19 [/SUP], Rodrigo Jiménez-Saiz[SUP] 20 21 22 23 [/SUP], Tilmann Kallinich[SUP] 24 [/SUP], Inge Kortekaas Krohn[SUP] 25 26 [/SUP], Charlotte G Mortz[SUP] 27 [/SUP], Carmen Riggioni[SUP] 28 [/SUP], Joaquin Sastre[SUP] 29 [/SUP], Milena Sokolowska[SUP] 30 31 [/SUP], Ziemowit Strzelczyk[SUP] 1 [/SUP], Eva Untersmayr[SUP] 32 [/SUP], Gerdien Tramper-Stranders[SUP] 33 34 [/SUP]; Immunology Section and Working Group Infections of the EAACI
Affiliations
Abstract
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.
Keywords: Delphi; MIS-C; SARS-CoV-2; children; clinical algorithm; clinical guidance; hyperinflammation; intravenous immunoglobulin; management; steroids.
. 2023 Jan;34(1):e13900.
doi: 10.1111/pai.13900.
Pathogenesis, immunology, and immune-targeted management of the multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multisystem syndrome (PIMS): EAACI Position Paper
Wojciech Feleszko[SUP] 1 [/SUP], Magdalena Okarska-Napierała[SUP] 2 [/SUP], Emilie Pauline Buddingh[SUP] 3 [/SUP], Marketa Bloomfield[SUP] 4 5 [/SUP], Anna Sediva[SUP] 4 [/SUP], Carles Bautista-Rodriguez[SUP] 6 7 [/SUP], Helen A Brough[SUP] 8 9 10 [/SUP], Philippe A Eigenmann[SUP] 11 [/SUP], Thomas Eiwegger[SUP] 12 13 14 15 [/SUP], Andrzej Eljaszewicz[SUP] 16 [/SUP], Stefanie Eyerich[SUP] 17 [/SUP], Cristina Gomez-Casado[SUP] 18 [/SUP], Alain Fraisse[SUP] 6 7 [/SUP], Jozef Janda[SUP] 19 [/SUP], Rodrigo Jiménez-Saiz[SUP] 20 21 22 23 [/SUP], Tilmann Kallinich[SUP] 24 [/SUP], Inge Kortekaas Krohn[SUP] 25 26 [/SUP], Charlotte G Mortz[SUP] 27 [/SUP], Carmen Riggioni[SUP] 28 [/SUP], Joaquin Sastre[SUP] 29 [/SUP], Milena Sokolowska[SUP] 30 31 [/SUP], Ziemowit Strzelczyk[SUP] 1 [/SUP], Eva Untersmayr[SUP] 32 [/SUP], Gerdien Tramper-Stranders[SUP] 33 34 [/SUP]; Immunology Section and Working Group Infections of the EAACI
Affiliations
- PMID: 36705045
- DOI: 10.1111/pai.13900
Abstract
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.
Keywords: Delphi; MIS-C; SARS-CoV-2; children; clinical algorithm; clinical guidance; hyperinflammation; intravenous immunoglobulin; management; steroids.