The genes associated with monogenic AIDs have been identified in recent years and, with the exception of MKD, which is caused by the deficiency of mevalonate kinase, the second enzyme of mevalonate/isoprenoid pathway, encode for proteins involved in the activity of inflammasome, a multiprotein complex which activates the processing and secretion of IL-1β and different other cytokines with proinflammatory effects [28]. Here, IL1B is linked to mevalonic aciduria.