Autoinflammatory diseases (AID) are defined according to the International Society of Auto-Inflammatory Diseases as abnormal activation of the innate immunity in the absence of infection or autoimmunity [1]. Familial Mediterranean Fever (FMF), associated with Pyrine inflammasome mutation, was the first monogenic AID described in 1997 [2,3]. FMF is the most frequent monogenic AID worldwide; patients display mutations in MEFV gene, which codes for the Pyrin protein [2,3]. Pyrin is part of a macromolecular complex called inflammasome and is expressed in innate immune cells, mainly monocytes and neutrophils [4,5].
