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
...efinitions and Medication...
...ble 1. Definitions for Selected Terms Used i...
Treatment and Diagnostic Testing
...and Diagnostic Testing...
...stic Testing and Treatment of Patients Wi...
...tice Statement: Intravenous immunog...
...ith acute KD who are at high risk of IVIG resistan...
...th acute KD who are at high risk of IVIG...
...nts with incomplete KD, prompt tre...
...patients with acute KD and suspected...
...patients with acute KD and persistent...
Ungraded Position Statement: For pa...
...th acute KD, using aspirin is strongly recommen...
...ith acute KD with subsequent resoluti...
...ents with acute KD with arthritis that...
...l diagnostic testing recommendations
...with suspected incomplete KD and fever, obtainin...
...or children with unexplained shock...
...th unexplained macrophage activation sy...
...gure 1. Key Recommendations for the Tre...