Kawasaki disease: etiopathogenesis and novel treatment strategies

Expert Rev Clin Immunol. 2017 Mar;13(3):247-258. doi: 10.1080/1744666X.2017.1232165. Epub 2016 Sep 13.


Kawasaki disease is an acute febrile systemic vasculitis that predominantly occurs in children below five years of age. Its etiopathogenesis is still not clear, but it is thought to be a complex interplay of genetic factors, infections and immunity. Areas covered: This review article discusses in detail Kawasaki disease, with particular emphasis on the recent updates on its pathogenesis and upcoming alternate treatment options. Though self-limiting in many cases, it can lead to severe complications like coronary artery aneurysms and thrombo-embolic occlusions, and hence requires early diagnosis and urgent attention to avoid them. Intravenous immunoglobulin (IVIG) with or without aspirin has remained the sole treatment option for these cases, but 10-15% cases develop resistance to this treatment. Expert commentary: There is a need to develop additional treatment strategies for children with Kawasaki disease. Targeting different steps of pathogenesis could provide us with alternate therapeutic options.

Keywords: Coronary artery aneurysm; Kawasaki disease; intravenous immunoglobulin; refractory Kawasaki disease; thrombo-embolic occlusion.

Publication types

  • Review

MeSH terms

  • Animals
  • Aspirin / therapeutic use*
  • Child, Preschool
  • Drug Resistance
  • Early Diagnosis
  • Gene-Environment Interaction
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Mucocutaneous Lymph Node Syndrome / diagnosis
  • Mucocutaneous Lymph Node Syndrome / etiology
  • Mucocutaneous Lymph Node Syndrome / therapy*
  • Remission, Spontaneous


  • Immunoglobulins, Intravenous
  • Aspirin