期刊
JOINT BONE SPINE
卷 75, 期 4, 页码 504-505出版社
ELSEVIER FRANCE-EDITIONS SCIENTIFIQUES MEDICALES ELSEVIER
DOI: 10.1016/j.jbspin.2008.04.001
关键词
anakinra; Familial Mediterranean Fever; colchicine
类别
Familial Mediterranean fever (FMF), a recessively inherited autoinflammatory disorder, is the prototype of a group of disorders termed systemic autoinflammatory diseases. Such diseases are characterized by seemingly unprovoked episodes of inflammation without evidence of high-titer autoantibodies or antigen-specific T cell. Repeated bouts of inflammation may lead to systemic AA protein deposition, making FMF a potentially fatal disease. Pyrin, the protein mutated in FMF, regulates caspase-1 activation and consequently IL-1 beta production. Although colchicine is the standard prophylactic therapy for attacks and amyloid deposition, some patients fail to respond or cannot tolerate its side effects. Anti-cytokine therapies have shown promise in the treatment of autoinflammatory disorders in children. We report on the use of the recombinant interleukin 1 receptor antagonist anakinra in one child with therapy-resistant FMF. The patient experienced immediate, sustained resolution of symptoms and laboratory markers of inflammation, and also, possibly, a reduced long-term risk of AA amyloidosis. (c) 2008 Elsevier Masson SAS. All rights reserved.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据