Kawasaki Disease
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
Definitions and Medi...
...itions for Selected Terms Used in the Recomm...
Treatment and Diagnostic Testing
...nt and Diagnostic Testi...
...iagnostic Testing and Treatment of Patien...
...ractice Statement: Intravenous immunoglo...
...ts with acute KD who are at high risk of...
...tients with acute KD who are at high risk...
...h incomplete KD, prompt treatment at time of dia...
...nts with acute KD and suspected or diagno...
...h acute KD and persistent fevers af...
...n Statement: For patients with acute...
...with acute KD, using aspirin is strongly rec...
...h acute KD with subsequent resolution...
...or patients with acute KD with arthritis that...
...iagnostic testing recommendations...
...dren with suspected incomplete KD and feve...
...with unexplained shock physiology, obtainin...
For children with unexplained macrophage activ...
...1. Key Recommendations for the Treatment of KD...