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Novel therapeutics for the treatment of familial Mediterranean fever: from colchicine to biologics.

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Familial Mediterranean fever (FMF), an inherited autosomal recessive disorder, is characterized by sporadic, paroxysmal attacks of fever and serosal inflammation, lasting 1-3 days. Patients may develop renal amyloidosis, arthritis, serositis, and skin and oral lesions. Diagnosis is based on clinical

Polyarteritis nodosa in case of familial Mediterranean fever.

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Gökçe İ, Altuntaş Ü, Filinte D, Alpay H. Polyarteritis nodosa in case of familial Mediterranean fever. Turk J Pediatr 2018; 60: 326-330. Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis,

Demographic, clinical and mutational characteristics of Turkish familial Mediterranean fever patients: results of a single center in Central Anatolia.

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Clinical and genetic findings of familial Mediterranean fever (FMF) may be variable in different populations. Environmental factors may also affect phenotypic features of FMF. In this study, we investigated demographic, clinical and mutational features of FMF patients who were treated in a single

Familial mediterranean Fever: diagnosing as early as 3 months of age.

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Familial Mediterranean Fever is an autosomal recessive disease. Major symptoms of disease are recurrent fever accompanied by serositis attacks. The disease is usually diagnosed before 20 years of age. Symptoms related to FMF are noted when children become more verbal, usually after 2 years of age.

Familial Mediterranean fever in small children in Turkey.

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OBJECTIVE Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterised by recurrent, self limited attacks of fever with serositis. The aim of our study was to describe the demographic, clinical and genetic features of FMF patients who had early disease onset and to compare

Neonatal onset familial Mediterranean fever.

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OBJECTIVE Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent, self-limited attacks of fever with serositis. Recently, it was shown that FMF patients with early disease onset have more severe disease. The aim of this study was to describe the demographic,

Efficacy and safety of canakinumab in adolescents and adults with colchicine-resistant familial Mediterranean fever.

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BACKGROUND This open-label pilot study aimed to investigate the efficacy of canakinumab in colchicine-resistant familial Mediterranean fever (FMF) patients. METHODS Patients with one or more attacks in a month in the preceding 3 months despite colchicine were eligible to enter a 30-day run-in

Twenty-Year Experience of a Single Referral Center on Pediatric Familial Mediterranean Fever: What Has Changed Over the Last Decade?

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Familial Mediterranean fever (FMF) is the most common autoinflammatory disease and is characterized by recurrent fever and serositis episodes. We aimed to share our 20-year FMF experience, clarify a phenotype-genotype correlation, and compare the characteristics and outcomes of

The contribution of genotypes at the MEFV and SAA1 loci to amyloidosis and disease severity in patients with familial Mediterranean fever.

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OBJECTIVE The clinical profile in familial Mediterranean fever (FMF), including its major manifestation, amyloidosis, is influenced by MEFV allelic heterogeneity and other genetic and/or environmental factors. In this study, we analyzed the contribution of genotypes at the MEFV and SAA1 loci to

Dysregulated production of interleukin-1β upon activation of the NLRP3 inflammasome in patients with familial Mediterranean fever.

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Familial Mediterranean fever (FMF) is caused by mutations in pyrin, a protein expressed in innate immune cells that interacts with caspase-1 and other inflammasome components to regulate interleukin (IL)-1β maturation. Since NLRP3 inflammasome represents major source of IL-1β, we studied its protein

The relation between familial Mediterranean fever and amyloidosis.

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Familial Mediterranean fever (FMF) is the most prevalent type of hereditary recurrent fever. Although the inflammatory attacks that characterize the disease may sometimes be debilitating, reactive amyloidosis remains the most serious manifestation of FMF. Daily treatment with colchicine can prevent

A microscopic study of kidney tissue in Familial Mediterranean Fever patients.

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Familial Mediterranean Fever (FMF) is an autosomal recessive hereditary disease leading mostly to renal failure and nephrotic syndrome. The ultrastructure of kidney has not been fully investigated in FMF associated renal disease. The aim of this study is to provide further evidence on the

[In vitro erythrophagocytosis in detecting opsonizing antibodies in a patient with Malta fever. A hypothesis on the explanation of in vivo hemophagocytosis in other patients with Malta fever].

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In vitro agglutination and phagocytosis of erythrocytes by homologous neutrophil granulocytes and monocytes could be observed using the blood of a healthy person (blood group A, Rh+) to detect opsonising antibodies in the serum of a patient with Malta fever (blood group O, Rh+). Agglutination and

Coexistence of Familial Mediterranean Fever With Ankylosing Spondylitis and Sjogren's Syndrome: A Rare Occurrence.

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Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent episodes of fever, peritonitis, synovitis, pleuritis, and erysipelas-like skin lesions. Sjogren's syndrome is a chronic autoimmune disorder characterized by dry eyes, dry mouth, and musculoskeletal involvement.

Familial Mediterranean fever: analysis of inheritance and current linkage data.

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Familial Mediterranean fever (FMF) is a genetic disorder characterized by recurrent attacks of fever and inflammation of serosal surfaces. Unlike many mendelian disorders, the mode of transmission has been subject to some controversy as segregation analysis studies have always demonstrated fewer

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