Pyrin is the pivotal protein of the related inflammasome, a member of cytosolic multiprotein oligomers family responsible for the activation of inflammatory responses in human cells. The Pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND) and familial Mediterranean fever (FMF) are well-known monogenic autoinflammatory diseases both related to GoF variants at different locus sites of the MEFV gene and associated with an excessive activation of the Pyrin inflammasome (no. 22 in Table 1 and Figure 1). Here, MEFV is linked to familial Mediterranean fever.