Finally the connection between pyrin and autoinflammation is observed in the Cryopyrin-Associated Periodic Syndromes (CAPS) in which mutations of genes encoding for the components of the proteins involved in the inflammasome (NLRP3) are implicated [25–27].The phenotypic heterogeneity characteristic of AI and AIF diseases, does not necessarily reflect fundamental genetic or mechanistic differences between these groups. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.