Bodar E J, van der Hilst J C H, Drenth J P H, van der Meer J W M, Simon A
Division of General Internal Medicine, Department of Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
Neth J Med. 2005 Jul-Aug;63(7):260-4.
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.
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.
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.
高IgD血症和周期性发热综合征（HIDS）是一种遗传性自身炎症性综合征，其特征为反复出现炎症发作。尽管辛伐他汀有益，依那西普可能有效，但HIDS的治疗仍很困难。研究一种罕见的周期性综合征的治疗因症状出现频率和严重程度各异以及患病率低而变得复杂。我们的目的是建立一个临床观察系统，以评估按需治疗的有效性。
对3例HIDS患者的7次发热发作进行了监测，监测期间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系统，其中包括12种症状。在1例患者中，疫苗接种引发了炎症发作。
在发作开始时，所有患者报告的临床评分为20至25分。该评分用于量化严重程度并确定发作结束。即使在这项初步研究中，对炎症发作进行可重复的监测也很困难。早期给予依那西普的效果不一。在1例患者中，疫苗接种可在12至24小时内轻易引发发热发作。在该患者中，IL-1ra类似物阿那白滞素在终止炎症发作方面比依那西普更成功。我们认为这种疫苗接种模型将允许在可控环境中评估按需治疗。
