FMF is a periodic fever syndrome characterised by recurrent bouts of fever with accompanying pain. It is typically inherited and the first symptoms of the flare usually occur before the age of 30.[1–5]
FMF is most prevalent in the eastern Mediterranean region and about 2.5 per 100,000 people are affected in Western countries. From a worldwide perspective, it is a rare disease.
FMF flares lasting from a few hours to 3-4 days can involve the following symptoms:[5,7,8]
However, fever may be the only symptom particularly during childhood.
In some cases it is unknown why FMF flares start, but the following have been identified as common triggers:
Possible symptoms of FMF (modified according to [3,4,7,8])
If FMF is left untreated, approximately 6-8 out of 10 patients will develop amyloidosis. Amyloidosis is a condition in which a protein called amyloid builds up in the kidneys leading to kidney failure. Improved diagnosis and treatment means that kidney damage caused by amyloidosis occurs less frequently than in the past.
In FMF, activation of the innate immune system and the inflammatory response occurs due to changes in the Mediterranean fever (MEFV) gene. To date, more than 300 different changes to this gene have been identified as possible causes of FMF.
 Samuels J, Ozen S. Curr Opin Rheumatol. 2006;18:108–17.
 Lachmann HJ, Hawkins PN. Arthritis Res Ther. 2009;11:212.
 Savic S, Dickie LJ, Battellino M et al. Curr Opin Rheumatol. 2012;24:103–12.
 Gattorno M, Federici S, Pelagatti MA et al. J Clin Immunol. 2008;28(Suppl 1):73–83.
 Manna R: Familial Mediterranean fever.
www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=342 (Last accessed on 13.10.2013).
 Fujikura K. Mol Genet Genomic Med. 2015;3:272–282.
 Ciccarelli F, De Martinis M, Ginaldi L. Curr Med Chem. 2014;21:261–269.
 Shohat M, Halpern GJ. Genet Med. 2011;13:487-498.
 MEFV sequence variants. Available from: http://fmf.igh.cnrs.fr/ISSAID/infevers (accessed November 2015).