Mutations in NLRP3 gene are known to cause cryopyrin-associated periodic fever syndromes (CAPS). NLRP3 gene encodes cryopyrin, a component of the NLRP3 inflammasome that activates caspase-1 leading to inflammation by excessive production of IL-1β [15, 25]. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.