title: Effects of the histone deacetylase inhibitor ITF2357 in autoinflammatory syndromes.
abstract: 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.
authors.affiliation: Department of General Internal Medicine and Nijmegen Institute for Infection, Inflammation, and Immunity (N4i), Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Evelien J
authors.initials: EJ
authors.lastname: Bodar
authors.forename: Anna
authors.initials: A
authors.lastname: Simon
authors.forename: Jos W M
authors.initials: JW
authors.lastname: van der Meer
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D005260:Female; D005334:Fever; D056660:Hereditary Autoinflammatory Diseases / Q000188:drug therapy*; D006801:Humans; D006877:Hydroxamic Acids / Q000627:therapeutic use*; D008297:Male; D054078:Mevalonate Kinase Deficiency / Q000188:drug therapy*; D008875:Middle Aged; D011446:Prospective Studies; D019873:Schnitzler Syndrome / Q000188:drug therapy; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D006877:Hydroxamic Acids; C502418:givinostat hydrochloride