A Comprehensive Update on Kawasaki Disease Vasculitis and Myocarditis

Curr Rheumatol Rep. 2020 Feb 5;22(2):6. doi: 10.1007/s11926-020-0882-1.


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.

Keywords: Anakinra; Coronary artery aneurysms; Interleukin-1; Intravenous immunoglobulins; Kawasaki disease; Myocarditis.

Publication types

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

MeSH terms

  • Animals
  • Anti-Inflammatory Agents / therapeutic use
  • Antirheumatic Agents / immunology
  • Antirheumatic Agents / therapeutic use
  • Coronary Aneurysm / etiology
  • Coronary Aneurysm / prevention & control*
  • Disease Models, Animal
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunosuppressive Agents / immunology
  • Immunosuppressive Agents / therapeutic use*
  • Infliximab / therapeutic use
  • Interleukin 1 Receptor Antagonist Protein / immunology
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use*
  • Mice
  • Mucocutaneous Lymph Node Syndrome* / diagnosis
  • Mucocutaneous Lymph Node Syndrome* / epidemiology
  • Mucocutaneous Lymph Node Syndrome* / etiology
  • Mucocutaneous Lymph Node Syndrome* / physiopathology
  • Myocarditis* / etiology
  • Myocarditis* / prevention & control
  • Vasculitis* / etiology
  • Vasculitis* / prevention & control


  • Anti-Inflammatory Agents
  • Antirheumatic Agents
  • Glucocorticoids
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Interleukin 1 Receptor Antagonist Protein
  • Infliximab