Familial Mediterranean Fever (FMF) is the most common hereditary autoinflammatory syndrome. It is caused by mutations in the MEFV gene which en- codes the pyrin protein, which has an important role in the activation of IL-1β (1). The condition manifests as attacks of serositis, com- monly involving the abdomen, chest or joints, typically accompanied by fever and elevated acute phase reac- tants. Colchicine is the standard drug used to prevent febrile attacks and the development of the most devas- tating complication of amyloidosis (2). Two recent articles describe: - the creation of outcome criteria that define and differentiate patients with FMF responsive and re- sistant to treatment; - the therapeutic efficacy of Canackinumab in col- chicine resistant FMF in childhood.
From the literature - FMF
GALLIZZI, Romina;
2014-01-01
Abstract
Familial Mediterranean Fever (FMF) is the most common hereditary autoinflammatory syndrome. It is caused by mutations in the MEFV gene which en- codes the pyrin protein, which has an important role in the activation of IL-1β (1). The condition manifests as attacks of serositis, com- monly involving the abdomen, chest or joints, typically accompanied by fever and elevated acute phase reac- tants. Colchicine is the standard drug used to prevent febrile attacks and the development of the most devas- tating complication of amyloidosis (2). Two recent articles describe: - the creation of outcome criteria that define and differentiate patients with FMF responsive and re- sistant to treatment; - the therapeutic efficacy of Canackinumab in col- chicine resistant FMF in childhood.Pubblicazioni consigliate
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