Clinical features of multisystem inflammatory syndrome in children

Curr Opin Rheumatol. 2021 Sep 1;33(5):378-386. doi: 10.1097/BOR.0000000000000818.

Abstract

Purpose of review: To review diagnosis, clinical characteristics and treatment of multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).

Recent findings: MIS-C emerged in spring 2020 as a hyperinflammatory syndrome following SARS-CoV-2 exposure in children. Despite growing awareness of MIS-C, diagnosis remains challenging due to the range of phenotypes and severity. Fever accompanied by shock, cardiac dysfunction, gastrointestinal symptoms, or mucocutaneous signs suggestive of Kawasaki disease, especially in the presence of known or suspected coronavirus disease 2019 exposure, should trigger consideration of MIS-C. However, clinical presentations are highly varied and may overlap with other infectious diseases. Clinicians must maintain a high index of suspicion for MIS-C and be aware that patients may develop coronary artery aneurysms and myocarditis even with few or no Kawasaki disease symptoms. More precise diagnostic criteria and specific biomarkers are needed to aid diagnosis. Intravenous immunoglobulin (IVIG) is first-line therapy, and steroids should be considered as initial adjunctive treatment for patients with severe manifestations or other risk factors. Prompt treatment is essential, as patients may worsen acutely, though overall prognosis is reassuring.

Summary: MIS-C associated with SARS-CoV-2 has varied clinical manifestations. Clinicians must be aware of the common presentation and potential for decompensation and cardiac sequalae to guide appropriate evaluation and treatment.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • COVID-19*
  • Humans
  • Mucocutaneous Lymph Node Syndrome* / diagnosis
  • Mucocutaneous Lymph Node Syndrome* / epidemiology
  • Mucocutaneous Lymph Node Syndrome* / therapy
  • SARS-CoV-2
  • Systemic Inflammatory Response Syndrome

Supplementary concepts

  • pediatric multisystem inflammatory disease, COVID-19 related