Introduction FMF is considered an autosomal recessive autoinflammatory syndrome caused by single gene (MEFV) mutations. Recently, it has been known that also heterozygous mutation carriers can suffer from a mild or incomplete form of FMF, named FMF-like disease. Among Armenians, who have relatively high carrier rate of MEFV mutations, single mutation has been detected in about 1/5 of symptomati...

Introduction Cutaneous Polyarteritis Nodosa (cPAN) is a rare type of vasculitis affecting small-to-medium-size arteries. It is distinct from systemic PAN in that it lacks significant internal organ involvement. Familial Mediterranean Fever (FMF) is the most common inherited autoinflammatory disease, characterized by recurrent, self–limited attacks of fever and aseptic polyserositis. PAN is cons...

OBJECTIVE Although the genetic etiology of familial Mediterranean fever (FMF) is known, limited information is available regarding the regulation of inflammation during attack-free periods. The aim of this study was to determine the alterations in serum copper (Cu), zinc (Zn) and selenium (Se) levels that may be associated with inflammation during attack-free periods in FMF patients. MATERIAL...

Introduction Autoinflammatory diseases are a group of disorders characterized by seemingly unprovoked inflammation in the absence of high-titer autoantibodies or antigen-specific T-cells. Familial Mediterranean fever (FMF) is the archetypal hereditary periodic fever syndrome and autoinflammatory disorder. It is characterized by recurrent selflimiting episodes of fever and painful polyserositis....

Background Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are most commonly employed acute phase reactants in follow up of patients with Familial Mediterranean Fever (FMF). As a rule CRP increases during FMF attacks but it returns to normal values in attack free periods. Persistently elevated acute phase reactants in attack free periods can be occasionally observed in patient...

Objectives FMF is an autosomal recessive disease, caused by mutations in the MEFV gene, encoding the pyrin protein associated with the interleukin-1b related inflammation cascade. In FMF overproduction of IL-1b and uncontrolled TNF-a release has been documented. These cytokines mediate activation of endothelial cells causing sustained inflammatory response and endothelial cell dysfunction[]. Va...

BACKGROUND Familial Mediterranean fever (FMF) is a disorder characterized by recurrent attacks of fever and serosal inflammation, particularly abdominal pain. Other disease processes, including medical and surgical emergencies, may mimic FMF, especially in atypical cases. CASE PRESENTATION We present a case of an adolescent male, referred to us with a diagnosis of colchicine resistant FMF, ul...

Results IL-1b, IL-6, IL-8, TNF-a, and IFN-g were not increased in either type of FMF in the remission state and in controls, and IL-6 was elevated during attack periods among patients. Serum IL-18 levels were significantly higher in typical FMF patients with M694I MEFV mutation in remission than in controls at the same level as flared Kawasaki disease, which further increased during attack peri...

Familial Mediterranean fever (FMF) is characterized by repeated episodes of fever, peritonitis, pleuritis, and synovitis. We describe here 3 Japanese patients (a mother and 2 children) in whom FMF was diagnosed on analysis of MEFV. A 40-year-old woman presented with fever and abdominal pain. The patient had had these symptoms on and off since childhood and consulted many hospitals. A 38-year-ol...

Familial Mediterranean fever (FMF) is an inherited periodic auto-inflammatory disease characterized by recurrent attacks of fever, synovitis and serositis. Ophthalmological manifestations of FMF are extremely rare. Here we described a boy who has been followed-up for FMF and attended with a loss of vision during the course of the disease. He was diagnosed with optic neuritis. As the other etiol...