Multisystem inflammatory syndrome in children: A dysregulated autoimmune disorder following COVID-19

J Microbiol Immunol Infect. 2023 Apr;56(2):236-245. doi: 10.1016/j.jmii.2023.01.001. Epub 2023 Jan 16.

Abstract

Multisystem inflammatory syndrome in children (MIS-C) is a dysregulated autoimmune-mediated illness in genetically susceptible patients following COVID-19 with an interval of 2-6 weeks. The median age of patients with MIS-C is 6-11 years. Most common manifestations are involvement of gastrointestinal tract, cardiovascular system, hematological system, and mucocutaneous system. Respiratory tract, neurological system, musculoskeletal system, and kidney are less frequently affected. Mucocutaneous manifestations and coronary artery abnormalities characteristic for Kawasaki disease (KD) may be observed in a significant proportion of MIS-C patients that may make the differential diagnosis be difficult for some patients, especially in the post-pandemic era. The mortality rate is 1-3%. Management and prognosis of MIS-C are similar to that of KD. MIS-C and KD may share a common pathogenic process. Based on the observation of MIS-C-like illness in uninfected neonates, i.e. multisystem inflammatory syndrome in neonates, both MIS-C and KD may be a consequence of dysregulated, over-exaggerated humoral immune responses triggered by a specific infectious agent.

Keywords: COVID-19; Kawasaki disease; Multisystem inflammatory syndrome in children.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases* / complications
  • Autoimmune Diseases* / diagnosis
  • COVID-19* / complications
  • Child
  • Diagnosis, Differential
  • Humans
  • Infant, Newborn
  • Mucocutaneous Lymph Node Syndrome* / complications
  • Mucocutaneous Lymph Node Syndrome* / diagnosis
  • Systemic Inflammatory Response Syndrome / diagnosis

Supplementary concepts

  • pediatric multisystem inflammatory disease, COVID-19 related