Diagnosis of FMF remains clinical and requires information about ethnic background, family history, and response to colchicine, since specific laboratory tests are not available [16]; genetic diagnosis can be confirmed by the presence of two mutations in the MEFV gene in at least 70% of patients with overt FMF, but heterozygous mutation carriers can also suffer from incomplete or even typical disease [17]. Here, MEFV is linked to familial Mediterranean fever.