Certain mutations in genes coding for key signaling proteins of innate immune pathways have been identified such as mutations of the MEFV gene coding for the pyrin protein in familial Mediterranean fever (FMF), the TNFRSF1A gene coding for the tumor necrosis factor receptor 1 protein in tumor necrosis factor receptor receptor-associated periodic syndrome (TRAPS), the MVK gene coding for mevalonate kinase in Hyper IgD syndrome (HIDS), and the NLRP3 gene coding for the NLRP3 protein in the cryopyrin-associated periodic syndromes (CAPS). Here, NLRP3 is linked to hyperimmunoglobulinemia D with periodic fever.