NLRP3-associated autoinflammatory disease (NLRP3-AID), often referred to as cryopyrin-associated periodic syndrome (CAPS), is a rare, heterogeneous disease entity caused by variants in the NLR family pyrin domain containing-3 (NLRP3) gene on chromosome 1q44 [1]. This evidence concerns the gene NLRP3 and cryopyrin-associated periodic syndrome.