FMF is traditionally seen as an autosomal-recessively inherited disorder caused by mutations in the MEFV gene located on chromosome 16p that encodes a 781 amino acid protein known as pyrin (or marenostrin) ([1] International FMF Consortium, Cell, 1997 [2]; French FMF Consortium, Nat Gen, 1997). Here, MEFV is linked to familial Mediterranean fever.