Introduction
Kawasaki Disease (KD) is an acute, self-limited febrile illness of unknown cause that predominantly affects children <5 years of age.1 Ithas been reported worldwide and is the leading cause of acquired heartdisease in children in developed countries.2The efficacy of intravenous immunoglobulin(IVIG)administered in the acute phase of KD is well established to reduce the prevalence of coronary artery abnormalities(CALs).3However, the approximately 10% to 20% of patients with KDwho do not respond to the initialtreatment with IVIGat least 36 hours after the end of their IVIG infusion are termed IVIG resistant.4It is wellknown that those with IVIG-resistant KD have a higher riskfor the development of CALs.5–6The treatment regimenfor IVIG resistant patients varies between institutions, and the best option has not yet beenestablished.7IVIG-resistant patients are usuallytreated with additional IVIG, the retrospective trials have tested efficacy, but IVIG retreatment has never been tested in an adequately randomized trial.8–9Thus, IVIG retreatment for IVIG-resistant KD is farfrom an established therapy.Corticosteroids have also been used to treat patients who have failed to respond to initial IVIG therapy for KD.10Several retrospective study results suggest thatcorticosteroid pulse therapy for IVIG-resistant KD may reduce the riskfor CALs.11–12There have been no prospective randomized controlled clinical trials(RCTs)comparing corticosteroid pulse therapy(CPT) with a second dose of IVIG for IVIG-resistant KD patients, and there are no robust data from clinical trials to guide the clinician in the choice of therapeutic agents for the child with IVIG resistance.13Therefore, we conducted a prospectiveRCT to determine whether patients with initial IVIG-resistant KD may benefit from CPT compared with additionalIVIG.