The best known HRF are Familiar Mediterranean Fever (FMF), Cryopyrin-Associated Periodic Syndrome (CAPS), TNF-receptor associated periodic fever syndrome (TRAPS) and Mevalonate-Kinase Deficiency (MKD), caused by mutations in MEFV, NLRP3, TNFRSF1A and MVK genes, respectively. Here, MVK is linked to cryopyrin-associated periodic syndrome.