FMF 无疑是遗传性复发性炎症疾病中最常见的疾病。该疾病主要影响地中海血统人群:来自东方和西方的阿拉伯人、亚美尼亚人、土耳其人、非德系和其他犹太人、德鲁兹人、黎巴嫩人、意大利人和希腊人。[10]Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet. 1998;351:659-664.http://www.ncbi.nlm.nih.gov/pubmed/9500348?tool=bestpractice.com在日本也报道了一些患者。在非德系犹太人和土耳其人中,一般人群中的杂合子频率可能大于 1/5。[10]Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet. 1998;351:659-664.http://www.ncbi.nlm.nih.gov/pubmed/9500348?tool=bestpractice.com[11]Gershoni-Baruch R, Brik R, Shinawi M, et al. The differential contribution of MEFV mutant alleles to the clinical profile of familial Mediterranean fever. Eur J Hum Genet. 2002;10:145-149.http://www.nature.com/ejhg/journal/v10/n2/full/5200776a.htmlhttp://www.ncbi.nlm.nih.gov/pubmed/11938447?tool=bestpractice.com高患病率解释了在这些人群中观察到的假显性遗传模式。非地中海血统人群患病率极低。[12]Tunca M, Ozdogan H. Molecular and genetic characteristics of hereditary autoinflammatory diseases. Curr Drug Targets Inflamm Allergy. 2005;4:77-80.http://www.ncbi.nlm.nih.gov/pubmed/15720239?tool=bestpractice.com[13]Tchernitchko D, Moutereau S, Legendre M, et al. MEFV analysis is of particularly weak diagnostic value for recurrent fevers in Western European Caucasian patients. Arthritis Rheum. 2005;52:3603-3605.http://onlinelibrary.wiley.com/doi/10.1002/art.21408/fullhttp://www.ncbi.nlm.nih.gov/pubmed/16255051?tool=bestpractice.com据报道,在美国境内的默伦琴人(欧洲、撒哈拉以南非洲、美洲原住民和地中海人的三种族混血后裔,大多数居住在阿巴拉契亚地区)中也有发生。
大多数患者 (90%) 在 20 岁前首次发作。在儿童中,20% 在 2 岁前首次发作,而 86% 在 10 岁前首次发作。[14]Majeed HA, Rawashdeh M, el-Shanti H, et al. Familial Mediterranean fever in children: the expanded clinical profile. QJM. 1999;92:309-318.http://qjmed.oxfordjournals.org/content/92/6/309.longhttp://www.ncbi.nlm.nih.gov/pubmed/10616706?tool=bestpractice.com
男性患者一般占 60%。然而,在患有淀粉样变性的患者中,男女比例为 2:1。[15]Gershoni-Baruch R, Brik R, Lidar M, et al. Male sex coupled with articular manifestations cause a 4-fold increase in susceptibility to amyloidosis in patients with familial Mediterranean fever homozygous for the M694V-MEFV mutation. J Rheumatol. 2003;30:308-312.http://www.ncbi.nlm.nih.gov/pubmed/12563686?tool=bestpractice.com