Cryopyrin-associated periodic syndromes: background and therapeutics.

Kubota T; Koike R

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Cryopyrin-associated periodic syndromes: background and therapeutics.

机译：隐索蛋白相关的周期性综合征：背景和治疗方法。

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Cryopyrin-associated periodic syndromes (CAPS) are caused by mutations of the gene encoding the NLR family protein NLRP3, which together with caspase-1 and adaptor proteins constitutes a protein complex termed the inflammasome. In innate immune reactions, a variety of stimuli activate the NLRP3 inflammasome, triggering caspase-1 to process proIL-1 and thus to produce mature IL-1. Excessive production of IL-1 by monocytes/macrophages is the central pathophysiology of CAPS, and daily injection of the IL-1 receptor antagonist anakinra rapidly ameliorates the inflammatory symptoms in most cases. Furthermore, double-blind, placebo-controlled clinical trials have recently confirmed the efficacy and safety of rilonacept, a fusion protein of the IL-1 receptor and IgG Fc, and canakinumab, a human anti-IL-1 monoclonal antibody, as novel long-lasting agents for treating CAPS.

机译：隐索蛋白相关的周期性综合征（CAPS）是由编码NLR家族蛋白NLRP3的基因突变引起的，该基因与caspase-1和衔接子蛋白一起构成了称为炎性体的蛋白复合物。在先天性免疫反应中，多种刺激激活NLRP3炎性体，触发caspase-1处理proIL-1，从而产生成熟的IL-1。单核细胞/巨噬细胞过量产生IL-1是CAPS的主要病理生理，在大多数情况下，每天注射IL-1受体拮抗剂anakinra可以迅速缓解炎症症状。此外，双盲，安慰剂对照的临床试验最近证实了IL-1受体与IgG Fc融合蛋白rilonacept和人抗IL-1单克隆抗体canakinumab的有效性和安全性。用于治疗CAPS的持久剂。

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《Modern rheumatology》 |2010年第3期|共9页
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Kubota T; Koike R;
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1. Cryopyrin-associated periodic syndromes: background and therapeutics. [J] . Kubota T, Koike R Modern rheumatology . 2010,第3期

机译：隐索蛋白相关的周期性综合征：背景和治疗方法。
2. Somatic NLRP3 mosaicism in Muckle-Wells syndrome. A genetic mechanism shared by different phenotypes of cryopyrin-associated periodic syndromes [J] . Nakagawa Kenji, Gonzalez-Roca Eva, Souto Alejandro, Annals of the Rheumatic Diseases: A Journal of Clinical Rheumatology and Connective Tissue Research . 2015,第3期

机译：Muckle-Wells综合征中的体细胞NLRP3镶嵌。冷冻蛋白相关周期性综合征不同表型共有的遗传机制
3. Cryopyrin-associated periodic syndromes (CAPS): report on two cases (Muckle-Wells syndrome (MWS) and CINCA/NOMID) [J] . SO Salugina, ES Fedorov, NN Kuzmina Pediatric rheumatology online journal . 2011,第S1期

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6. Systematic literature review of efficacy/effectiveness and safety of current therapies for the treatment of cryopyrin-associated periodic syndrome hyperimmunoglobulin D syndrome and tumour necrosis factor receptor-associated periodic syndrome [O] . Jasmin Beate Kuemmerle-Deschner, Raju Gautam, Aneesh Thomas George, 2020

机译：系统文献综述疗效/有效性和安全性的疗效治疗过渡相关周期综合征超引治蛋白D综合征和肿瘤坏死因子受体相关的周期综合征
7. Guidelines for the management and treatment of periodic fever syndromes Cryopyrin-associated periodic syndromes (cryopyrinopathies – CAPS) [O] . Terreri Maria Teresa R.A., Bernardo Wanderley Marques, Len Claudio Arnaldo, 2016

机译：定期发烧综合症的治疗和治疗指南隐索蛋白相关的周期性综合症（隐孢子虫病– CAPS）

Cryopyrin-associated periodic syndromes: background and therapeutics.

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