Kawasaki disease (KD) is the most common systemic vasculitis syndrome, primarily affecting small- to medium-sized arteries, more particularly the coronary arteries (Kato et al. 1996). KD was first described in 1967 and is now identified as the leading cause of acquired heart disease among children in developed countries (Wang et al. 2005). The annual incidence of KD in children of Japanese descent is about 218 per 100,000 children less than 5 years of age (Nakamura et al. 2012) as compared to about 20 per 100,000 in the United States (Holman et al. 2010a). Timely treatment with high-dose intravenous γ globulin (IVIG) reduces the duration of fever and incidence of coronary artery lesions (CAL). However, even after IVIG treatment ∼5–7% of patients develop aneurysms (Ogata et al. 2013).