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Review
. 2021;9(4):83-92.
doi: 10.1007/s40124-021-00257-6. Epub 2021 Oct 19.

Current Insights Into the Pathophysiology of Multisystem Inflammatory Syndrome in Children

Laura A Vella  1   2 , Anne H Rowley  3   4
Affiliations
Review

Current Insights Into the Pathophysiology of Multisystem Inflammatory Syndrome in Children

Laura A Vella et al. Curr Pediatr Rep. 2021.

Abstract

Purpose of review: We highlight the new clinical entity multisystem inflammatory syndrome in children (MIS-C), the progress in understanding its immunopathogenesis, and compare and contrast the clinical and immunologic features of MIS-C with Kawasaki disease (KD).

Recent findings: Studies show immune dysregulation in MIS-C including T lymphocyte depletion and activation, T cell receptor Vbeta skewing, elevated plasmablast frequencies, increased markers of vascular pathology, and decreased numbers and functional profiles of antigen-presenting cells.

Summary: MIS-C is a late manifestation of infection with SARS-CoV-2 associated with marked immune activation and many potential mechanisms of immunopathogenesis. MIS-C and KD have clinical similarities but are distinct. Myocardial dysfunction with or without mild coronary artery dilation can occur in MIS-C but generally corrects within weeks. In contrast, the coronary arteries are the primary target in KD, and coronary artery sequelae can be lifelong. Supportive care and anti-inflammatory therapy appear to hasten improvement in children with MIS-C, and there is hope that vaccines will prevent its development.

Keywords: Inflammatory syndrome; Kawasaki disease; MIS-C; Pediatric; SARS-CoV-2.

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Figures

Fig. 1
Fig. 1
Potential immunopathogenesis of MIS-C. Created with Biorender.com
See this image and copyright information in PMC

References

    1. • Bailey LC, Razzaghi H, Burrows EK, et al (2021) Assessment of 135794 pediatric patients tested for severe acute respiratory syndrome coronavirus 2 across the United States. JAMA Pediatr 175:176–184. Reports a lack of association between MIS-C and KD diagnoses. - PMC - PubMed
    1. •Whittaker E, Bamford A, Kenny J, et al (2020) Clinical characteristics of 58 children with a pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2. JAMA. 10.1001/jama.2020.10369. Describes clinical features of children in Europe with MIS-C early in the pandemic. - PMC - PubMed
    1. • Godfred-Cato S, Bryant B, Leung J, et al (2020) COVID-19-associated multisystem inflammatory syndrome in children - United States, March-July 2020. MMWR Morb Mortal Wkly Rep 69:1074–1080. Analyzes clinical differences between acute COVID-19 and MIS-C. - PMC - PubMed
    1. Feldstein LR, Rose EB, Horwitz SM, et al (2020) Multisystem inflammatory syndrome in U.S. children and adolescents. N Engl J Med. 10.1056/NEJMoa2021680. Reports clinical features of children in the USA with MIS-C.
    1. • Campbell JI, Roberts JE, Dubois M, Naureckas Li C, Sandora TJ, Lamb GS (2021) Non-SARS-CoV-2 infections among patients evaluated for MIS-C associated with COVID-19. Pediatr Infect Dis J 40:e90–e93. Demonstrates the wide differential diagnosis of MIS-C. - PMC - PubMed

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