Familial Mediterranean Fever (FMF) is an autosomal recessive inherited disease characterized by recurrent attacks of fever and pain. The disease follows a course with fever accompanied with peritonitis, pleuritis and arthritis. Typically, attacks accompanied by fever and serositis continue for 1 to 4 days with spontaneous healing being observed. Renal failure due to amyloidosis is the most serious complication of the disease. The disease is very common, especially in Turkish, Armenian, Arab and Jewish communities, as well as the Ashkenazi. In these communities, the incidence of the disease may increase to as much as one in 200 with a carrier frequency of one in five.
The disease is caused by mutations in the MEFV gene. The MEFV encodes the ‘pyrin’ protein. The MEFV encodes the broad ‘pyrin’ protein. The gene mainly manifests itself in granulocytes and is responsible for bringing inflammation under control. To date, up to 180 mutations have been identified in the MEFV gene.