The major breakthroughs in the understanding of autoinflammatory diseases came around the turn of the century with the identification of the genetic cause for several periodic fever syndromes, including familial Mediterranean fever (FMF) due to pathogenic variants in MEFV and cryopyrin associated periodic syndrome (CAPS) due to pathogenic variants in NLRP3, affecting the pyrin and NLRP3 inflammasome function, respectively, and leading to dysregulation of IL-1 production [3,4,5] Since that time, there has been a flurry of discovery in the autoinflammatory disorder field [6]. Here, NLRP3 is linked to familial Mediterranean fever.