Multisystem Inflammatory Syndrome in Children: Unique Disease or Part of the Kawasaki Disease Spectrum?

Caterina Matucci-Cerinic; Roberta Caorsi; Alessandro Consolaro; Silvia Rosina; Adele Civino; Angelo Ravelli

doi:10.3389/fped.2021.680813

Multisystem Inflammatory Syndrome in Children: Unique Disease or Part of the Kawasaki Disease Spectrum?

Front Pediatr. 2021 Jun 4:9:680813. doi: 10.3389/fped.2021.680813. eCollection 2021.

Authors

Caterina Matucci-Cerinic¹, Roberta Caorsi², Alessandro Consolaro^{1

2}, Silvia Rosina², Adele Civino³, Angelo Ravelli^{1

2

4}

Affiliations

¹ Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili, Università degli Studi di Genova, Genoa, Italy.
² Unità Operativa Complessa (UOC) Clinica Pediatrica e Reumatologia, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Giannina Gaslini, Genoa, Italy.
³ Unità di Reumatologia e Immunologia Pediatrica, Ospedale Vito Fazzi, Lecce, Italy.
⁴ Sechenov First Moscow State Medical University, Moscow, Russia.

Abstract

One of the most intriguing and mysterious phenomena observed during the COVID-19 pandemic has been represented by the occurrence of the multisystem inflammatory syndrome in children and adolescents (MIS-C). Patients with this condition have some overlapping signs and symptoms with those of Kawasaki disease (KD), but also display clinical features that are uncommon or less frequent in this illness, such as diarrhea, abdominal pain and myocardial involvement. The sickest patients may develop multiorgan failure and shock, usually due to myocarditis. Management is based on the administration of intravenous immunoglobulin, glucocorticoids and, in the most severe instances, anakinra. It is still debated whether MIS-C and KD represent different illnesses or are part of the same disease spectrum. The aim of the present review is to analyze critically the evidence in favor of the latter hypothesis and to provide the authors' personal interpretation of the relationship between the two conditions.

Keywords: COVID-19; Kawasaki disease; MIS-C; SARS-CoV-2; macrophage activation syndrome; multisystem inflammatory syndrome in children; toxic shock syndrome.