Among the already recognized autoinflammatory disorders are autosomal dominant diseases related to NLRP3 (CIAS1) mutations, including chronic infantile neurologic cutaneous articular syndrome (CINCA) and Muckle-Wells syndrome (MWS), and the autosomal recessive (AR) familial Mediterranean fever (FMF) due to MEFV mutations. Here, MEFV is linked to Muckle-Wells syndrome.