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...
...1. Definitions for Selected Terms Used in the Rec...
Treatment and Diagnostic Testing
...Treatment and...
...Table 3. Diagnostic Testi...
...Practice Statement: Intravenous im...
...atients with acute KD who are at high risk...
...r patients with acute KD who are at high risk...
...or patients with incomplete KD, prompt t...
...with acute KD and suspected or diagnosed macrop...
...tients with acute KD and persistent fevers a...
...Position Statement: For patients with acu...
...s with acute KD, using aspirin is strongly...
...ents with acute KD with subsequent...
...tients with acute KD with arthritis t...
...Additional di...
...en with suspected incomplete KD and fev...
...th unexplained shock physiology, obtaining...
...en with unexplained macrophage activatio...
Figure 1. Key Recommendations for...