Familial mediterranean fever overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Familial mediterranean fever is a hereditary inflammatory disorder that affects groups of patients originating from around the Mediterranean Sea (hence its name). It is prominently present in the Armenian people (up to 1 in 7 affected), Sephardi Jews (and, to a much lesser extent, Ashkenazi Jews), people from Turkey, the Arab countries and Lebanon.[1]
Historical Perspective
A New York allergist, Dr Sheppard Siegal, first described the attacks of peritonitis in 1945; he termed this "benign paroxysmal peritonitis", as the disease course was essentially benign.[2] Dr Hobart Reimann, working in the American University in Beirut, described a more complete picture which he termed "periodic disease".[3]
Causes
Familial mediterranean fever is most often caused by a mutation in the MEFV gene. This gene creates proteins involved in inflammation.
References
- ↑ Livneh A, Langevitz P. Diagnostic and treatment concerns in familial Mediterranean fever. Baillieres Best Pract Res Clin Rheumatol 2000;14(3):477-98. PMID 10985982.
- ↑ Siegal S. Benign paroxysmal peritonitis. Ann Intern Med 1945;23:1-21.
- ↑ Reiman HA. Periodic disease. Probable syndrome including periodic fever, benign paroxysmal peritonitis, cyclic neutropenia and intermittent arthralgia. JAMA 1948;136:239-44.