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transmission of blood-borne pathogens. Elaborate sterilization procedures, including
lyophilization, pasteurization, and addition of solvent detergents, are generally effective
in rendering the product free of infectious agents. There have been no cases of
infections transmitted by IVIG since current purification practices were initiated in
1995. Overall, significant toxicity is rare, and benefits clearly outweigh risks in
children with confirmed KD.
Aspirin
Aspirin was the first medication to be used for treatment of KD, both for its antiinflammatory and its antithrombotic effects. High-dose (>80 mg/kg/day) and low-dose
regimens (30 mg/kg/day) in four divided doses are still used in conjunction with IVIG
during the acute phase of the illness despite the fact that meta-analyses demonstrate no
additive protection from coronary artery aneurysms from aspirin. Once fever has
resolved for 48 hours, patients are generally switched to antiplatelet doses of aspirin (3
to 5 mg/kg/day). Unless coronary artery abnormalities are detected by echocardiogram,
aspirin is discontinued once laboratory studies return to normal, usually within 2
months of the onset of KD.
The risks of aspirin appear to be similar to those reported in other settings:
transaminitis, chemical hepatitis, transient hearing loss, and, rarely, Reye syndrome.
These risks may even be increased in KD: Aspirin-binding studies have suggested that
the hypoalbuminemia of children with KD predisposes them to toxic levels of free
salicylate despite measured (bound) levels within the therapeutic range. At least one
case of Reye syndrome has been reported after 6 days of aspirin therapy for KD.
Alternative antipyretic and anti-inflammatory agents, such as ibuprofen, may be used
for treatment of arthralgias, and aspirin should be rapidly discontinued whenever
intercurrent illness with varicella or influenza is possible. Influenza vaccine should be
given to the patient and household contacts at the time of diagnosis according to
seasonal recommendations. For those patients who are unable to tolerate aspirin, other
antiplatelet agents (e.g., clopidogrel) should be considered.
Adjunctive Therapy With Corticosteroids
New evidence has emerged supporting the use of glucocorticoids for the primary
treatment of KD in patients who carry risk factors that could lead to resistance with
IVIG. In 2017, the AHA recognized that use of adjunctive therapy with glucocorticoids

may be beneficial for patients considered to be at high risk for developing CAA and/or
at increased risk of resistance to IVIG. Among Japanese children, there are clearly
delineated criteria to define those patients as high-risk. For non-Japanese children,
there are no validated criteria to identify children before treatment. Some experts
recommend adding glucocorticoids to non-Japanese children with the following
criteria: enlarged CAA at presentation (LAD or RCA CA z score of ≥2.5 on baseline
echo), age ≤6 months, Kawasaki shock syndrome, and KD presenting with MAS.