Highlights & Basics
- Familial Mediterranean fever is primarily a genetic disease due to Mendelian-recessive inheritance of Mediterranean fever gene mutations.
- Occurs mainly in people of Mediterranean ancestry, especially from Arab countries, Turkey, Israel, and Armenia.
- Characterized by recurrent attacks of fever and systemic inflammation, typically lasting 24-72 hours and presenting in childhood.
- Rare manifestations include chronic arthritis, spondyloarthropathy, myopathies, and protracted febrile myalgia.
- Clinical judgment is important in establishing the diagnosis, which is often made retrospectively.
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Definition
Epidemiology
Etiology
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Citations
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Giancane G, Ter Haar NM, Wulffraat N, et al. Evidence-based recommendations for genetic diagnosis of familial Mediterranean fever. Ann Rheum Dis. 2015 Apr;74(4):635-41.[Abstract]
Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016 Apr;75(4):644-51.[Abstract]
Kallinich T, Haffner D, Niehues T, et al. Colchicine use in children and adolescents with familial Mediterranean fever: literature review and consensus statement. Pediatrics. 2007 Feb;119(2):e474-83.[Abstract]
Hentgen V, Grateau G, Kone-Paut I, et al. Evidence-based recommendations for the practical management of familial Mediterranean fever. Semin Arthritis Rheum. 2013 Dec;43(3):387-91.[Abstract]
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