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Causes
By Mayo Clinic staff
The cause of familial Mediterranean fever is a gene defect on chromosome 16. The gene, called MEFV, carries the genetic code for a protein called pyrin, which is involved in regulating inflammation. More than 50 different mutations in the MEFV gene have been identified. Although it's not certain exactly how this defect causes familial Mediterranean fever, it may be that people with this mutation don't make pyrin, hence, inflammatory responses aren't regulated and may be quite severe.
Familial Mediterranean fever is inherited as an autosomal recessive trait, meaning that you must inherit the mutated gene from each parent in order to develop the condition.
- Familial Mediterranean fever. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/print/sec19/ch297/ch297b.html. Accessed May 28, 2010.
- Kastner DL. Familial Mediterranean fever. In: Fauci AS, et al. Harrison's Principles of Internal Medicine. 17th ed. New York, N.Y.: McGraw-Hill; 2008. http://www.accessmedicine.com/content.aspx?aID=2899961. Accessed May 28, 2010.
- Fonnesu C, et al. Familial Mediterranean fever: A review for clinical management. Joint Bone Spine. 2009;76:227.
- Rosenberg PM, et al. Clinical manifestations and diagnosis of familial Mediterranean fever. http://www.uptodate.com/home/index.html. Accessed June 1, 2010.
- Galip G, et al. Current perspectives on familial Mediterranean fever. Current Opinion in Infectious Diseases. 2009;22:309.
- Rosenberg PM, et al. Management of familial Mediterranean fever. http://www.uptodate.com/home/index.html. Accessed June 1, 2010.
- Ben-Chetrit E, et al. Familial Mediterranean fever in the world. Arthritis and Rheumatism. 2009;61:1447.
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