Molecular Medicine

, Volume 17, Issue 5–6, pp 363–368 | Cite as

Effects of the Histone Deacetylase Inhibitor ITF2357 in Autoinflammatory Syndromes

  • Evelien J Bodar
  • Anna Simon
  • Jos W M van der Meer
Research Article


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.



Italfarmaco SpA, Cinisello Balsamo (Milano), Italy, prepared the ITF2357 capsules. A Simon received a Netherlands Organisation for Health Research and Development (ZonMW) VENI grant and is now a recipient of a ZonMW VIDI grant.


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Copyright information

© The Feinstein Institute for Medical Research 2011

Authors and Affiliations

  • Evelien J Bodar
    • 1
  • Anna Simon
    • 1
  • Jos W M van der Meer
    • 1
  1. 1.Department of General Internal Medicine and Nijmegen Institute for Infection, Inflammation, and Immunity (N4i), Internal post 463Radboud University Nijmegen Medical CentreHB Nijmegenthe Netherlands

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