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Abstract

Familial Mediterranean Fever (FMF) is an autosomal recessive inherited disease, which is accompanied by recurrent attacks of fever and serositis. It can be distinguished into two types. Type 1, is associated with recurrent short episodes of inflammation and polyserositis; type 2, is characterized by the accumulation of serum amyloid A mainly in the kidney leading to amyloidosis. The etiology of this disease is due to mutations in the MEFV gene, which encodes the protein “pyrin”. These mutations cause the uncontrolled production of proinflammatory cytokines including interleukin 1. Genetic analysis is important to confirm the diagnosis in the patients. Colchicine is the drug of choice. However, some people are resistant to this drug. In such cases, newer biologic agents have used in the treatment of the disease. This review aims to discuss the most recent advances about FMF including the major symptoms, the diagnosis, the genetics and the management.

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