Mutations within NLRP3 and Pyrin lead to the development of autoinflammatory diseases, including cryopyrin-associated periodic syndromes (CAPS) and familial Mediterranean fever (FMF).14–18 Additionally, chronic inflammasome activation has been tied to the development of metabolic syndromes, neurodegenerative diseases, and cancer progression.19–21 It is therefore unsurprising that inflammasome activation is a tightly regulated process. Here, MEFV is linked to familial Mediterranean fever.