Randomized clinical trials have established that treatment within the first 10 days of onset of fever with IVIG (2 g/kg) and aspirin (80-100 mg/kg/day) reduces the risk of coronary artery damage and aneurysm formation from 20% to 25% to only 3% to 5% (52). Approximately 85% of patients will respond to a single dose of IVIG with cessation of fever and disappearance of all the clinical signs of the vasculitis. There is much speculation, but little data, on the mechanism of action of IVIG in acute KD. Possible mechanisms include cross-linking of FcgII and FcgIII receptors on macrophages, selective induction of IL-1-receptor antagonist and IL-8, or provision of specific antiagent, anti-idiotype, anticytokine, or antitoxin antibodies (53,54). Approximately 15% of KD patients will fail to respond to a single dose of IVIG and have persistent fever (55). The optimal treatment regimen for these patients has not been determined by randomized, controlled trials. These patients are at higher risk of coronary artery aneurysm formation and should receive additional immunomodulatory therapy, which may include a second dose of IVIG, high-dose pulse methylprednisolone (30 mg/kg/day x 3 days), or infliximab (5 mg/kg) (56-58).
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