Familial mediterranean fever: literature review and clinical case

Abstract

Familial Mediterranean fever is the most common monogenic autoinflammatory disease in the world. More
than 100 thousand patients with this disease are registered in the world. It has now been proven that FMF is
caused by mutations in the MEFV gene, located on the short arm of chromosome 16. The disease has a distinct
ethnic predisposition and is manifested by recurrent attacks of febrile fever lasting 6–72 hours, accompanied
by symptoms of aseptic peritonitis, pleurisy, arthritis, and inflammatory rash. The severity of the disease is
determined not only by attacks of systemic inflammation, which significantly worsen the quality of life, but also
by a high risk of developing AA amyloidosis, which can lead to renal failure and death of the patient.