Familial Mediterranean Fever: a review for clinical management

Joint Bone Spine. 2009 May;76(3):227-33. doi: 10.1016/j.jbspin.2008.08.004. Epub 2008 Dec 16.


Familial Mediterranean Fever (FMF) is a hereditary autosomal recessive, autoinflammatory disorder characterized by recurrent, self-limiting episodes of short duration (mean 24-72 h) of fever and serositis. FMF is the most frequent periodic febrile syndrome among the autoinflammatory syndromes (AS), a heterogeneous group of recently identified diseases clinically characterized by recurrent febrile attacks, in the absence of autoantibodies and antigen-specific T lymphocytes. In FMF, periodic attacks show inter- and intra-individual variability in terms of frequency and severity. Usually, they are triggered by apparently innocuous stimuli and may be preceded by a prodromal period. The Mediterranean FeVer gene (MEFV) responsible gene maps on chromosome 16 (16p13) encoding the pyrin-marenostrin protein. The precise pathologic mechanism is still to be definitively elucidated; however a new macromolecular complex, called inflammasome, seems to play a major role in the control of inflammation and it might be involved in the pathogenesis of FMF. The most severe long-term complication is type AA amyloidosis, principally affecting the kidney and the cause of chronic renal failure. Two types of risk factors, genetic and non-genetic, have been identified for this complication. Currently, the only effective treatment of Familial Mediterranean Fever is the colchicine. New drugs in a few colchicine resistant patients have been tried, but additional studies on larger series are necessary to draw definitive conclusions.

Publication types

  • Review

MeSH terms

  • Amyloidosis / complications
  • Colchicine / therapeutic use
  • Cytoskeletal Proteins / genetics
  • Familial Mediterranean Fever / complications
  • Familial Mediterranean Fever / genetics
  • Familial Mediterranean Fever / rehabilitation*
  • Gout Suppressants / therapeutic use
  • Humans
  • Kidney Failure, Chronic / complications
  • Pyrin


  • Cytoskeletal Proteins
  • Gout Suppressants
  • MEFV protein, human
  • Pyrin
  • Colchicine