Familial mediterranean fever: a prevalent condition amongest the arabs

ABSTRACT

Autoinflammatory diseases are a group of disorders characterized by seemingly unprovoked inflammation in the absence of high-titer autoantibodies or antigen-specific T-cells and include the hereditary periodic fever syndromes. Familial Mediterranean Fever [FMF] is the prototype of the autoinflammatory disorder. It is an autosomal recessive disorder with high prevalence in non Ashkenazi Jews, Armenians, Turks and Arabs. The classic clinical picture is recurrent acute short-lived febrile and painful attacks with variable periods of remission. It is complicated by amyloidosis that leads to renal failure in a subset of patients. The gene responsible for FMF, MEFV, has been identified and its role in inflammation is under study. There appears to be a distinctive clinical picture among Arab FMF patients and the spectrum and distribution of MEFV mutations is different from other ethnic groups commonly affected by FMF. The clinical and molecular aspects of FMF in the Arabs are discussed