Interim guidance on Kawasaki disease and acute multisystem inflammatory syndrome in children and adolescents in the current emergency scenario of SARS-CoV-2 infection

Version May 21, 2020

ISS COVID-19 Rare Diseases Working Group

Domenica Taruscio, Istituto Superiore di Sanità, Roma Luigi Bertinato, Istituto Superiore di Sanità, Roma Claudio Carta, Istituto Superiore di Sanità, Roma Marta De Santis, Istituto Superiore di Sanità, Roma Paola Meli, Istituto Superiore di Sanità, Roma Paolo Salerno, Istituto Superiore di Sanità, Roma Marco Salvatore, Istituto Superiore di Sanità, Roma Marco Silano, Istituto Superiore di Sanità, Roma In collaboration with: ISS COVID-19 Drugs Working Group Monica Boirivant, Istituto Superiore di Sanità, Roma Patrizia Popoli, Istituto Superiore di Sanità, Roma ISS COVID-19 Infection prevention and control Working Group Fortunato Paolo D’Ancona, Istituto Superiore di Sanità, Roma and in collaboration with: Rolando Cimaz, Università degli Studi di Milano D’Antiga Lorenzo, Ospedale Giovanni XXIII, Bergamo Alessandra Marchesi, Ospedale Pediatrico Bambino Gesù, Roma. Angelo Ravelli, Istituto “Giannina Gaslini”, Genova Alberto Villani, Ospedale Pediatrico Bambino Gesù, Roma. Presidente Società di Pediatria Christian Pescosolido, Associazione Malattia di Kawasaki “Rari ma Speciali Odv” Ministero della Salute, Direzione generale della prevenzione sanitaria, Ufficio “Prevenzione delle malattie trasmissibili e profilassi internazionale” Anna Caraglia Francesco Paolo Maraglino Patrizia Parodi English version edited by Alessandra Marchesi and Laura Lee Cellai. The responsibility for scientific and technical data lies with the authors, who declare that they do not have any conflict of interest. Editing and graphics: ISS Scientific Communication Unit (Sandra Salinetti and Paola De Castro) © Istituto Superiore di Sanità 2020 viale Regina Elena, 299 –00161 Roma Istituto Superiore di Sanità Interim guidance on Kawasaki disease and acute multisystem inflammatory syndrome in children and adolescents in the current emergency scenario of SARS-CoV-2 infection. Version of May 21, 2020. ISS COVID-19 Rare Diseases Working Group 2020, 14 p. Rapporto ISS COVID-19 n. 29/2020 - English version

The available scientific evidence indicates that the clinical course of SARS-CoV-2 infection that occurs in pediatric patients has very low lethality rates (0.06% in the age group 0-15 years). However, recent European and US publications describe an acute multisystem inflammatory syndrome in children and adolescents, associated with positivity for SARS-CoV-2 or to the presence of antibodies antiSARS-CoV-2, the precise classification of which is currently in process. This syndrome seems to share some clinical features with KD, such as an aberrant inflammatory response and some therapeutic options (immunoglobulins, steroids, anti-cytokine drugs). The syndrome, however, could be distinguished from KD by other peculiarities, represented by the age of the affected subjects, severe multisystemic involvement, prevalent myocardial and / or gastrointestinal involvement. The document highlights that, at the moment, even in the absence of a European shared case definition, a correlation between SARS-CoV-2 infection and the onset of the syndrome is plausible, even if there is limited evidence of the causal link. It also stresses the absence of strong epidemiological evidence of an increased incidence of KD, as well as the absence of increased risk of disease recurrence and the absence of increased susceptibility to SARS-CoV-2 infection in patients with previous KD during the COVID-19 pandemic. The original Italian version of ISS COVID-19 Reports are available from: https://www.iss.it/rapporti-covid-19 The reports translated in English are available from: https://www.iss.it/rapporti-iss-covid-19-in-english For information: domenica.taruscio@iss.it

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