To date, 138 sequence variants of NLRP3 have been identified, of which 110 have been associated with CAPS, and 28 are either nonpathogenic or of undetermined significance [13], the commonest of which is the Q703K variant (also described as Q705K in older literature) [14] that has been reported in 5% of healthy Caucasian alleles [11]. This evidence concerns the gene NLRP3 and cryopyrin-associated periodic syndrome.