Gain-of-function mutations of NLRC4 lead to spontaneous inflammasome activation, giving rise to pediatric enteritis and recurrent macrophage activation syndrome (MAS).19,20 Notably, our recent research has unveiled that NLRC4 activation by the rod protein of the Escherichia coli T3SS triggers disseminated intravascular coagulation (DIC), contributing to host mortality subsequent to caspase-1 activation and pyroptosis.21 In alignment with these findings, patients with NLRC4-MAS also exhibit coagulation abnormalities.22 Here, CASP1 is linked to macrophage activation syndrome.