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Familial Mediterranean Fever

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Auto-Inflammatory Syndromes

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Abstract

Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease which has a high prevalence in the eastern Mediterranean. The typical FMF attack is characterized by fever and polyserositis in the form of abdominal or chest pain or arthritis along with high acute phase reactants. FMF is caused by different mutations in the MEFV gene coding for pyrin. The gain-of-function mutations in the MEFV gene are associated with an exaggerated inflammatory response via an increased production of interleukin-1.

An international group of experts has published the recommendations for the management of FMF. Colchicine is still the mainstay of FMF treatment, and its regular use prevents attacks, controls subclinical inflammation, and decreases the development of amyloidosis which is the most significant complication of FMF. However, a minor proportion of patients can be resistant to colchicine. If colchicine fails, anti-IL-1 therapy is a promising second-line therapy.

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Authors and Affiliations

  1. Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey

    Selcan Demir & Seza Özen

  2. Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey

    İlkin Elif Günel

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  1. New York University School of Medicine, New York, NY, USA

    Petros Efthimiou

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Demir, S., Günel, İ.E., Özen, S. (2019). Familial Mediterranean Fever. In: Efthimiou, P. (eds) Auto-Inflammatory Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-319-96929-9_9

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