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.
Diagnosis
History and Symptoms
The diagnosis is clinically made on the basis of the history of typical attacks, especially in patients from the ethnic groups in which FMF is more highly prevalent.
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.