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.