Activating mutations in several inflammasome sensor proteins, such as NLRP3, NLRC4, and MEFV, result in autoinflammatory conditions that respond favorably to the inhibition of IL-1 (29), and in the case of NLRC4 mutations, the clinical course of disease can resemble HLH (30, 31). This evidence concerns the gene IL1B and hemophagocytic syndrome.