Rheumatology International

, Volume 26, Issue 6, pp 489–496

Familial Mediterranean fever

Review

DOI: 10.1007/s00296-005-0074-3

Cite this article as:
Onen, F. Rheumatol Int (2006) 26: 489. doi:10.1007/s00296-005-0074-3

Abstract

Familial Mediterranean fever (FMF) is the most frequent hereditary inflammatory disease characterized by self-limited recurrent attacks of fever and serositis. It is transmitted in an autosomal recessive pattern and affects certain ethnic groups mainly Jews, Turks, Arabs, and Armenians. FMF is caused by mutations in MEFV gene, which encodes pyrin. This protein is expressed mainly in myeloid/monocytic cells and modulates IL-1β processing, NF-κB activation, and apoptosis. A mutated pyrin probably results in uncontrolled inflammation. The most devastating complication of FMF is amyloidosis, leading to chronic renal failure. M694V homozygocity, male gender and the α/α genotype of serum amyloid A1 gene are the currently established risk factors for development of amyloidosis. Daily colchicine is the mainstay of the therapy for the disease, resulting in complete remission or marked reduction in the frequency and duration of attacks in most patients. It is also effective in preventing and arresting renal amyloidosis.

Keywords

Familial Mediterranean fever

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  1. 1.Division of Immunology and Rheumatology, Department of Internal MedicineDokuz Eylul University School of MedicineBalcova-IzmirTurkey