Intravenous immunoglobulin for the treatment of Kawasaki disease

Standard first-line therapy for Kawasaki disease (KD) consists of intravenous immunoglobulin (IVIG) and aspirin. Current guidelines recommend 2 g/kg of IVIG and 80 to 100 mg/kg of aspirin administered within the first 10 days of illness. This regimen has marked efficacy in preventing the development of coronary artery aneurysms. Approximately 15% to 20% of treated patients require a second dose of IVIG to control the inflammatory process. The role of adjunctive corticosteroid therapy with IVIG and aspirin is evolving, with Japanese studies showing a clear benefit in those patients at highest risk for development of coronary disease. The challenge in North America has been reliable identification of the highest-risk patients, which still eludes us because the Japanese scoring systems are ineffective in multiethnic populations. Despite its efficacy, the precise mechanism of IVIG’s effect in KD is unclear but probably relates to its ability to down-regulate aspects of the up-regulated inflammatory response in patients with KD.