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10.1080/2162402X.2017.1318237

http://scihub22266oqcxt.onion/10.1080/2162402X.2017.1318237
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C5486186!5486186!28680751
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suck abstract from ncbi


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pmid28680751      Oncoimmunology 2017 ; 6 (6): ä
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  • Tocilizumab in patients with multisystem Erdheim?Chester disease #MMPMID28680751
  • Berti A; Cavalli G; Guglielmi B; Biavasco R; Campochiaro C; Tomelleri A; Nicoletti R; Panzacchi A; Ferrarini M; Dagna L
  • Oncoimmunology 2017[]; 6 (6): ä PMID28680751show ga
  • Treatment of Erdheim?Chester disease (ECD), a rare non-Langerhans histiocytosis, relies on interferon-?, chemotherapeutic agents such as purine analogs, cytokine-blocking agents and BRAF inhibitors. Since interleukin (IL)-6 levels are elevated in serum and lesions of ECD patients, we evaluated the therapeutic efficacy and safety of IL-6 blockade with tocilizumab. We conducted an open-label, single-arm, phase II, prospective study of tocilizumab in three patients with multisystem ECD and poor tolerance/contraindications to IFN-?. Modifications of symptoms attributed to ECD represented the criteria for evaluation of clinical response. Changes at positron emission tomography scan, computed tomography scan, and magnetic resonance imaging at month 6 represented the main criteria for the evaluation of radiological response.Sustained complete clinical response and partial radiological improvement were observed in two patients, paralleled by modulation of systemic pro-inflammatory mediators. In spite of disease stabilization or improvement at extra-neurological sites, a third patient experienced a radiologic and clinical progression of central nervous system involvement, mirrored by a dramatic increase of circulating IL-6 and related cytokines. These findings indicate that IL-6 inhibition can be effective in ECD, but caution is advisable in patients with neurologic involvement. IL-6 emerges as a central mediator in ECD pathogenesis.
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