Numbers around the disease
Populations with high prevalence of the disease (1:200-1:1,000) are non-Ashkenazi Jews, Turks, Armenians and Arabs. The disease is also common in Italy, Greece, and Spain.
Usually the disease appears before the age of 30. An earlier start is often associated with more severe symptoms.
A distinction is made between 2 types in the FMF:
- Type 1 of the FMF is characterized by:
- Type 2 of the FMF is characterized by:
The Tel-Hashomer criteria require for diagnosis that two major criteria (fever and serositis, amyloidosis AA, efficacy of colchicine) and two minor criteria (recurrent fever attacks, erysipelas-like erythema, relatives with FMF) are present. The positive predictive value of a genetic analysis is only 70-80%.