Epidemiology

FMF is the most frequent hereditary recurrent inflammatory disorder. This disease affects mainly populations of Mediterranean descent: Arabs from the East as well as from the West, Armenians, Turks, non-Ashkenazi and other Jews, Druzes, Lebanese, Italians, and Greeks.[11][12] Patients have also been reported in Europe, North America, and Japan.[13] Turkey and Armenia have the highest estimated FMF prevalence, at 1:1000 and 1:500, respectively.[12]

The reported gene carrier frequency is 1:5 in non-Ashkenazi Jews, 1:16 in Arabs, 1:5 in Turks, and 1:7 in Armenians.[12] High prevalence explains the pseudo-dominant mode of inheritance observed in these populations. Prevalence is extremely rare among people of non-Mediterranean descent.[14][15] There are also reports of occurrence in Melungeon populations (tri-racial mix of European, sub-Saharan African, American Indian, and Mediterranean origins living mostly in the Appalachian region) within the US.

The majority of patients (80% to 90%) have their first attack before age 20 years.[13] Among paediatric populations, 20% have their first attack before age 2 years and 86% before age 10 years.[16]

Males usually comprise 60% of patients. Among those with amyloidosis, the male to female ratio is 2:1.[17]

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