Effect of etanercept and anakinra on inflammatory attacks in the hyper-IgD syndrome: introducing a vaccination provocation model.
Effect of etanercept and anakinra on inflammatory attacks in the hyper-IgD syndrome: introducing a vaccination provocation model.
复制标题
依那西普和阿那白滞素对高 IgD 综合征炎症发作的影响：引入疫苗接种激发模型。
DOI：
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发表时间：
2005
影响因子：
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通讯作者：
A. Simon
中科院分区：
文献类型：
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作者：
E. Bodar;J. C. H. Hilst;J. Drenth;J. Meer;A. Simon
背景
高IgD和周期性发热综合征（HIDS）是一种遗传性自身炎症综合征，其特征是反复的炎症发作。HIDS的治疗是困难的，虽然辛伐他汀是有益的，依那西普可能是有效的。研究一种罕见的周期性综合征的治疗是复杂的不同频率和症状的严重程度和低患病率。我们的目标是开发一个临床观察系统，以评估按需治疗的有效性。
方法
监测了3例HIDS患者的7次发热发作，有或没有给予依那西普或阿那白滞素。我们制定了一个临床评分，其中包括12个症状。在一名患者中，炎症发作是由疫苗接种引起的。
结果和结论
在发作时，所有患者报告的临床评分在20至25之间。该分数用于量化严重程度并定义攻击的结束。即使在这项初步研究中，对炎症发作的可重复监测也很困难。依那西普早期给药的影响是可变的。在一名患者中，接种疫苗可在12至24小时内容易地引起发热发作。在该患者中，IL-1 ra类似物阿那白滞素在中止炎症发作方面比依那西普更成功。我们建议，这种疫苗接种模型将允许在受控环境中评估按需治疗。
BACKGROUND
Hyper-IgD and periodic fever syndrome (HIDS) is an hereditary autoinflammatory syndrome, characterised by recurrent inflammatory attacks. Treatment of HIDS is difficult, although simvastatin is beneficial and etanercept might be effective. Studying the treatment of a rare periodic syndrome is complicated by the varying frequency and severity of symptoms and low prevalence. Our aim was to develop a system of clinical observations to evaluate effectiveness of treatment-on-demand.
METHODS
Seven fever episodes in three HIDS patients were monitored, with and without administration of etanercept or anakinra. We developed a clinical score, which includes 12 symptoms. In one patient, inflammatory attacks were provoked by vaccination.
RESULTS AND CONCLUSIONS
At the onset of an attack, all patients reported a clinical score between 20 and 25. The score was used to quantify severity and define the end of an attack. Reproducible monitoring of inflammatory episodes was difficult, even in this pilot study. The effect of early administration of etanercept was variable. In one patient, a fever episode could be readily provoked within 12 to 24 hours by vaccination. In this patient, the IL-1ra analogue anakinra was more successful in aborting the inflammatory attack than etanercept. We propose that this vaccination model will allow evaluation of treatment-on-demand in a controlled setting.