Severe COVID-19, multisystem inflammatory syndrome in children, and Kawasaki disease: immunological mechanisms, clinical manifestations and management

Rheumatol Int. 2021 Jan;41(1):19-32. doi: 10.1007/s00296-020-04749-4. Epub 2020 Nov 21.

Abstract

Multisystem inflammatory syndrome (MIS-C) is a pediatric hyperinflammation disorder caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). It has now been reported from several countries the world over. Some of the clinical manifestations of MIS-C mimic Kawasaki disease (KD) shock syndrome. MIS-C develops 4-6 weeks following SARS-CoV-2 infection, and is presumably initiated by adaptive immune response. Though it has multisystem involvement, it is the cardiovascular manifestations that are most prominent. High titres of anti-SARS-CoV-2 antibodies are seen in these patients. As this is a new disease entity, its immunopathogenesis is not fully elucidated. Whether it has some overlap with KD is still unclear. Current treatment guidelines recommend use of intravenous immunoglobulin and high-dose corticosteroids as first-line treatment. Mortality rates of MIS-C are lower compared to adult forms of severe COVID-19 disease.

Keywords: Coronavirus disease 2019; Hyperinflammation; Kawasaki disease (KD); Kawasaki-like disease; Multisystem inflammatory syndrome (MIS-C).

Publication types

  • Review

MeSH terms

  • COVID-19 / diagnosis
  • COVID-19 / physiopathology*
  • COVID-19 / therapy
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage
  • Mucocutaneous Lymph Node Syndrome / diagnosis
  • Mucocutaneous Lymph Node Syndrome / physiopathology*
  • Pandemics
  • SARS-CoV-2
  • Systemic Inflammatory Response Syndrome / diagnosis
  • Systemic Inflammatory Response Syndrome / physiopathology*
  • Systemic Inflammatory Response Syndrome / therapy

Substances

  • Immunoglobulins, Intravenous

Supplementary concepts

  • pediatric multisystem inflammatory disease, COVID-19 related