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
...tions and Medications
...e 1. Definitions for Selected Terms...
Treatment and Diagnostic Testing
...t and Diagnostic Testing...
...agnostic Testing and Treatment of Patients With KD...
...Practice Statement: Intravenous immunoglobulin...
...or patients with acute KD who are at...
...r patients with acute KD who are at hi...
...h incomplete KD, prompt treatment at time of...
...patients with acute KD and suspected or diagnose...
...or patients with acute KD and persistent...
...ition Statement: For patients with acute KD and p...
...patients with acute KD, using aspirin is strong...
For patients with acute KD with subsequ...
...or patients with acute KD with arthritis tha...
...iagnostic testing recommendations...
...n with suspected incomplete KD and f...
...with unexplained shock physiology, obtain...
...h unexplained macrophage activation...
...e 1. Key Recommendations for the T...