Figure 2: Clinical criteria (Tel Hashomer criteria) for the diagnosis of Familial Mediterranean fever (FMF). The clinical diagnosis of FMF is considered definitive in the presence of least two major criteria, or one major plus two minor criteria. The diagnosis of FMF is probable in the presence of one major criterion plus one minor criterion. Adapted from Livneh et al. Criteria for the diagnosis of Familial Mediterranean Fever. Arthritis Rheum. 1997; 40:1879–85 [76]. After identification of the first mutations in MEFV in 1997 [34,57], a role for genetic testing must be considered during the diagnostic workup.