Kawasaki disease (KD) is an acute childhood disease with coronary artery lesions.
Endothelial cell injury is mediated by T cells and cytokines (TNF-α and IL-1β).
KD is due to an abnormal immunological response in genetically predisposed individuals.
Candidate KD susceptibility genes identified by GWAS include BLK, CD40 and FCGR2A.
Intravenous immunoglobulin and aspirin are the standard treatment of acute KD.
IVIG-resistant patients are treated with steroids, cyclosporine, anti-IL-1β or anti-TNF.