Kawasaki Disease

Publication Date: March 6, 2022

Key Points

Key Points

  • Kawasaki disease (KD) is a medium vessel vasculitis, one of two primary common vasculitis syndromes that predominantly affect children (the other being Henoch-Schönlein purpura/IgA vasculitis).
    • KD typically presents in children under 5 years of age with an incidence in the US of 25–50/100,000, similar to that of type 1 diabetes.
    • KD treated appropriately is associated with a low rate of mortality (approximately 0.08% case fatality rate in the largest series).
    • However, in the developed world, KD is the most common cause of acquired cardiac disease in childhood, with 25% of untreated and 5% of treated cases developing coronary artery aneurysms.
    • In infants less than 6 months of age, the risk of a coronary artery aneurysm is 50% even in KD patients treated with intravenous immunoglobulin (IVIG) within the first 10 days of illness.
  • These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and role of echocardiography for patients with suspected or confirmed KD.

Definitions and Medications

...tions and Medications...

...le 1. Definitions for Selected Terms Used...


Treatment and Diagnostic Testing

...atment and Diagnostic Testing

...Diagnostic Testing and Treatment of Patient...

...d Practice Statement: Intravenous immunoglo...

...h acute KD who are at high risk of IVIG resistan...

...h acute KD who are at high risk of IVIG resistan...

...with incomplete KD, prompt treatment at t...

...ents with acute KD and suspected or diagnosed mac...

...ith acute KD and persistent fevers after i...

...aded Position Statement: For patients with a...

...with acute KD, using aspirin is strongly...

...r patients with acute KD with subsequent resolu...

...nts with acute KD with arthritis that persist...

...diagnostic testing recommendatio...

...n with suspected incomplete KD and f...

...th unexplained shock physiology, obtai...

...ildren with unexplained macrophage activation...


...ecommendations for the Treatment of KD...