Table 1

 Comparison of clinical and genetic criteria of familial Mediterranean fever

ReferenceStudy n*Clinical criteria†Genetic criterion‡Sensitivity (%)§Specificity (%)¶
FMF, familial Mediterranean fever; MEFV, Mediterranean fever; ND, not done.
*n, number in the total population, followed in brackets by the number of patients in the subgroups from (the four mainly affected ethnic groups; patients from other origin).
†Clinical criteria as defined by Livneh.
‡Genetic criterion as defined by the identification of at least two MEFV mutations.
§Percentage of MEFV-positive patients among those with a clinical FMF.
¶Percentage of MEFV-negative patients among those negative for the clinical criteria.
Samuels et al2100 (52; 48)Yes246228100
Tchernitchko et al24208 (0; 208)Yes02080ND
This study1574 (752; 822)Yes409 (351; 58)709 (235; 474)37 (60; 11)97 (91.6; 99.7)
No15 (14; 1)441 (152; 289)