CAPS are associated with upregulated interleukin (IL) 1β production due to mutations in the nucleotide-binding oligomerisation domain (NOD)-like receptor family, pyrin domain containing 3 (NLRP3) gene.4NLRP3 encodes cryopyrin, a key inflammasome component that triggers caspase-1 enzyme activation, which in turn catalyses the cleavage of pro-IL-1β into active IL-1β, a potent proinflammatory and pyrogenic cytokine. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.