These disorders arise from an improper control of inflammation, due to mutations in inflammasome-related proteins (such as pyrin in FMF, cryopyrin and NLRP12 in FCAS), or to defect in other proteins indirectly leading to abnormal inflammasome activation (such as mevalonate kinase in MKD and TNF-receptor in TRAPS) [3]. This evidence concerns the gene MVK and mevalonic aciduria.