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A Comprehensive Update on Kawasaki Disease Vasculitis and Myocarditis

  • Pediatric Rheumatology (S Ozen, Section Editor)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Purpose of the Review

Kawasaki disease (KD) is a childhood systemic vasculitis of unknown etiology that causes coronary artery aneurysms (CAA), and if left undiagnosed can result in long-term cardiovascular complications and adult cardiac disease. Up to 20% of KD children fail to respond to IVIG, the mainstay of therapy, highlighting the need for novel therapeutic strategies. Here we review the latest findings in the field regarding specific etiology, genetic associations, and advancements in treatment strategies to prevent coronary aneurysms.

Recent Findings

Recent discoveries using the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model have accelerated the study of KD pathophysiology and have advanced treatment strategies including clinical trials for IL-1R antagonist, Anakinra.

Summary

KD remains an elusive pediatric vasculitis syndrome and is the leading cause of acquired heart disease among children in the USA and developed countries. Advancements in combination treatment for refractory KD with further understanding of novel genetic risk factors serve as a solid foundation for future research endeavors in the field.

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Funding

The work of M.A. is supported by the NIH Grant R01 AI072726 and M.N.R. is supported by the NIH grant R01 HL139766.

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Authors and Affiliations

  1. Division of Pediatric Infectious Diseases and Immunology; Burns and Allen Research Institute, Cedars-Sinai Medical Center and David Geffen School of Medicine at UCLA, Los Angeles, CA, 90048, USA

    Priya R. Soni, Magali Noval Rivas & Moshe Arditi

  2. Department of Biomedical Sciences—Infectious and Immunologic Diseases Research Center (IIDRC), Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA

    Magali Noval Rivas & Moshe Arditi

  3. Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA

    Moshe Arditi

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  1. Priya R. Soni
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Correspondence to Moshe Arditi.

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Soni, P.R., Noval Rivas, M. & Arditi, M. A Comprehensive Update on Kawasaki Disease Vasculitis and Myocarditis. Curr Rheumatol Rep 22, 6 (2020). https://doi.org/10.1007/s11926-020-0882-1

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