To date, mutations in cell death regulatory genes, including TNFR1, XIAP, A20, OTULIN, LUBAC components (HOIL-1, HOIP and SHARPIN), RIPK1, and caspase-8, cause primary immunodeficiency, autoimmunity, and/or trigger autoinflammation reminiscent of NLRP3 autoactivating mutation-associated cryopyrin-associated periodic syndromes (CAPS). The gene discussed is NLRP3; the disease is cryopyrin-associated periodic syndrome.