Effects of the Histone Deacetylase Inhibitor ITF2357 in Autoinflammatory Syndromes

被引：0
作者：
Evelien J Bodar
Anna Simon
Jos W M van der Meer
机构：
[1] Radboud University Nijmegen Medical Centre,Department of General Internal Medicine and Nijmegen Institute for Infection, Inflammation, and Immunity (N4i), Internal post 463
来源：
Molecular Medicine | 2011年 / 17卷
关键词：
Autoinflammatory Syndrome; HDAC Inhibitor ITF2357; Tumor Necrosis Factor Receptor-associated Periodic Syndrome (TRAPS); Schnitzler Syndrome; TRAPS Patient;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
We explored the effects of the oral histone deacetylase (HDAC) Inhibitor ITF2357 In patients with autoinflammatory syndrome. In this prospective open-label pilot study, eight patients were enrolled; one patient with tumor necrosis factor receptor-associated periodic syndrome (TRAPS), three patients with hyper-IgD and periodic fever syndrome (HIDS) and four patients with Schnitzler syndrome were closely followed during 90 d of ITF2357 treatment. Three patients with Schnitzler syndrome and one TRAPS patient experienced a partial remission. In four patients, there was no effect. In HIDS patients, there was a tendency toward a higher attack frequency and increasing attack severity. In two patients (one TRAPS and one HIDS), we observed a decrease of acute-phase response without signs of clinical improvement. One patient with Schnitzler syndrome showed a partial response despite an ongoing acute-phase response. In conclusion, ITF2357 monotherapy was able to induce partial response only in patients with Schnitzler syndrome and no response in patients with HIDS.
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页码：363 / 368
页数：5
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