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A Comprehensive Update on Kawasaki Disease Vasculitis and Myocarditis


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.


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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Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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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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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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  • Kawasaki disease
  • Coronary artery aneurysms
  • Myocarditis
  • Intravenous immunoglobulins
  • Interleukin-1
  • Anakinra