Patients who fulfill the criteria for Kawasaki disease (KD) or incomplete KD require treatment because of the risk of cardiovascular complications that may result in significant morbidity and mortality．
In patients with KD, we recommend a single dose of intravenous immune globulin (IVIG; 2 g/kg) administered over 8 to 12 hours (Grade 1A). It is preferable that IVIG be administered within the first 10 days of illness, before aneurysms typically develop, but IVIG should be administered even beyond this 10-day window in patients with evidence of persistent vasculitis or systemic inflammation (eg, persistent fever).
In patients with KD, we suggest that aspirin be administered in the acute phase of illness (Grade 2C). The American Academy of Pediatrics (AAP) and American Heart Association (AHA) recommend high-dose aspirin (80 to 100 mg/kg/day), but it is not clear that this dose is more effective than the lower doses used in some clinical trials (30 to 50 mg/kg per day). The total daily aspirin dose of 30 to 50 mg/kg per day is administered in four divided doses (maximum dose 4 g per day). The dose of aspirin is decreased to 3 to 5 mg/kg per day 48 hours after the resolution of fever. Aspirin is continued until laboratory markers of acute inflammation (eg, platelet count and erythrocyte sedimentation rate [ESR]) return to normal, unless coronary artery (CA) abnormalities are detected by echocardiography.
Glucocorticoids may more rapidly reduce the level of inflammatory markers and fever and decrease the rate of initial treatment failure in certain high-risk patients. However, the addition of glucocorticoids to initial IVIG therapy does not impact the overall rate of CA aneurysms. Thus, we do not recommend routine use of glucocorticoids for initial therapy of KD (Grade 1B).
Prognosis is based upon the severity of CA involvement as a marker of risk for myocardial infarction. After the baseline echocardiogram is obtained at diagnosis, echocardiography is usually repeated at approximately two and six weeks of illness to evaluate for CA involvement. Guidelines have been developed by the AHA and AAP for subsequent therapy, physical activity, and follow-up visits (schedule and content) based upon the relative risk for myocardial infarction.
Patients without any cardiovascular abnormalities appear to be clinically healthy at long-term follow-up (range, 10 to 21 years). However, it is unknown whether they are at increased risk for atherosclerotic heart disease.
We suggest postponing administration of live-virus vaccines (eg, measles, varicella) for at least 11 months in children who have been treated with IVIG because IVIG can interfere with vaccine immunogenicity (Grade 2C). One exception to this postponement is in children residing in communities experiencing an outbreak of a vaccine-preventable disease. Another exception is children on long-term aspirin therapy. We suggest that children who are ≥6 months of age and are on long-term aspirin therapy receive varicella and inactivated influenza vaccines because of the possible increased risk of Reye syndrome (Grade 2C).