NLRP3 and cryopyrin-associated periodic syndrome: Mutations in NLRP3 gene are described in approximately 60% of CAPS patients, causing the constitutive activation of the inflammasome and dysregulation with IL-1 overproduction; excessive IL-1 signaling appears to be a constant feature in the background of CAPS, driven by gain-of-function NLRP3 mutations, even in the absence of a second signal (58).