As aforementioned, CINCA/NOMID represents the most severe phenotype of CAPS, monogenic diseases due to autosomal dominant inheritance of mutations in the NLRP3 gene (formerly known as cold-induced autoinflammatory syndrome 1, CIAS1), which encodes the NLRP3 protein also termed cryopyrin. This evidence concerns the gene NLRP3 and CINCA syndrome.