Fig. 8: Schematic model for endothelial cell pyroptosis in Kawasaki disease.

High level of serum HMGB1 interacts with its receptor RAGE, enters lysosomes, and induces Cat B (cathepsin B) activation and release from the ruptured lysosomes. This is followed by NLRP3 inflammasome activation and subsequent induction of caspase-1-dependent pyroptosis