Familial mediterranean fever in Arabs.
Autoinflammatory diseases are a group of disorders characterised by seemingly unprovoked inflammation in the absence of high-titre autoantibodies or antigen-specific T cells, and include the hereditary periodic fever syndromes. Familial Mediterranean fever (FMF) is an archetypal autoinflammatory disorder, which is autosomal recessive and has a 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. In a subset of patients, the disorder is complicated by amyloidosis that leads to renal failure. The gene responsible for FMF--MEFV--has been identified and its role in inflammation is being assessed. There seems to be a distinctive clinical picture in Arab patients with FMF, and the range and distribution of MEFV mutations is different from that noted in other affected ethnic groups. Here, we discuss the clinical and molecular aspects of FMF in Arabs.
El-Shanti
Hatem
H
Department of Pediatrics, Division of Medical Genetics, University of Iowa, Iowa City, IA, USA. hatem-el-shanti@uiowa.edu
Majeed
Hasan Abdel
HA
El-Khateeb
Mohammed
M
D000686:Amyloidosis / Q000150:complications; D018912:Arabs; D002648:Child; D003078:Colchicine / Q000627:therapeutic use; D003598:Cytoskeletal Proteins / Q000235:genetics; D010505:Familial Mediterranean Fever / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D005260:Female; D006074:Gout Suppressants / Q000627:therapeutic use; D006801:Humans; D008297:Male; D009154:Mutation; D010641:Phenotype; D000071198:Pyrin; D012008:Recurrence; D051437:Renal Insufficiency / Q000209:etiology
D003598:Cytoskeletal Proteins; D006074:Gout Suppressants; C000604192:MEFV protein, human; D000071198:Pyrin; D003078:Colchicine