Other examples of inflammasome-regulating genes and associated autoinflammatory diseases include MVK (hyper-IgD syndrome; HIDS), NLRP3 (Cryopyrin-associated periodic fever syndrome; CAPS), PSTPIP1 (Pyogenic arthritis with pyoderma gangrenosum and acne; PAPA), WDR1 (periodic fever, immunodeficiency, and thrombocytopenia; PFIT), IL1RA (Deficiency of IL-1RA; DIRA), and NLRC4 (Macrophage activation syndrome; MAS). This evidence concerns the gene IL1R1 and hyperinsulinemic hypoglycemia, familial, 4.