Since some patients with gain-of-function mutations in NLRP12 exhibit symptoms similar to those of CAPS, the disease was named FCAS2 [102] and patients with NALP12 periodic fever syndrome respond to canakinumab (an anti-human IL-1β monoclonal antibody) and/or etanercept (a tumor necrosis factor (TNF) receptor-IgG heavy chain chimeric protein that acts as a bivalent antagonist of TNF activity) [102], the pathogenesis of NLRP12 autoinflammatory syndrome (NLRP12-AD) may explain the gain of function of the NLRP12 inflammasome by a similar mechanism of the NLRP3 inflammasome (Fig. 2). This evidence concerns the gene NLRP12 and autoinflammatory syndrome.