ABSTRACT

Hereditary recurrent fever are a group of inherited systemic disorders characterized by episodes of fever with a variety of localized inammatory manifestations such as fever, diarrhea, abdominal pain, rash, arthralgia, or arthritis. Familial Mediterranean fever (FMF, MEFV) is the prototype of this family of diseases. The other hereditary recurrent fevers include the tumor necrosis factor (TNF) receptor-associated periodic syndrome, hyperimmunoglobulinemia D with periodic fever syndrome, familial cold autoinammatory syndrome, Muckle-Wells syndrome, and neonatal onset multisystem inammatory disease. It is accepted that FMF is a recessively inherited disorder in individuals of non-Ashkenazi Jewish, Armenian, Arab, and Turkish ancestry (Eisenstein et al. 2013). The gene responsible for FMF was rst reported in 1997 (Eisenstein et al. 2013). It is characterized by episodes of fever with localized inammation, often affecting serosal membranes, joints, and skin (Eisenstein et al. 2013). Between the fever episodes, patients with most of these syndromes generally feel healthy and function normally (Eisenstein et al. 2013).