Objective. To establish a new set of criteria for the diagnosis of fam
ilial Mediterranean fever (FMF), Methods. Twenty-seven features and ma
nifestations typical of FMF were studied to determine their prevalence
in 105 patients with FMF and 106 controls. Diagnosis of FMF in the st
udy group was based on clinical judgment. Controls were patients with
a variety of other diseases who presented to the emergency room or out
patient clinics with recurrent episodes of pain in body sites usually
involved in FMF attacks, Manifestations observed to be significantly m
ore common in FMF patients than in controls were incorporated into the
rule proposed for diagnosis of FMF, based on a model of major, minor,
and supportive criteria, Results. Two sets of diagnostic criteria wer
e established. A conservative criteria set for diagnosis of FMF was ba
sed on the presence of 1 major or 2 minor criteria, or 1 minor plus 5
supportive criteria, and a simple criteria set for diagnosis of FMF re
quired 1 major or 2 minor criteria, The sensitivity and specificity of
these 2 criteria sets were >95% and >97%, respectively. Conclusion. T
he proposed new sets of criteria were highly sensitive and specific, a
nd could be used to readily diagnose FMF and to distinguish FMF from o
ther periodic febrile diseases.