Tocilizumab in patients with multisystem Erdheim-Chester disease
| Autor: | B. Guglielmi, Alessandro Tomelleri, Giulio Cavalli, Alvise Berti, Corrado Campochiaro, Roberto Nicoletti, Andrea Panzacchi, Riccardo Biavasco, Lorenzo Dagna, Marina Ferrarini |
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| Přispěvatelé: | Berti, Alvise, Cavalli, Giulio, Guglielmi, Barbara, Biavasco, Riccardo, Campochiaro, Corrado, Tomelleri, Alessandro, Nicoletti, Roberto, Panzacchi, Andrea, Ferrarini, Marina, Dagna, Lorenzo |
| Jazyk: | angličtina |
| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty genetic structures Immunology Erdheim–Chester disease Disease non-langerhans cell histiocytosis Gastroenterology ErdheimâChester disease 03 medical and health sciences chemistry.chemical_compound Non-Langerhans cell histiocytosis tocilizumab 0302 clinical medicine Tocilizumab non-Langerhans cell histiocytosi Internal medicine non-Langerhans cell medicine Immunology and Allergy Prospective cohort study Interleukin 6 RC254-282 medicine.diagnostic_test biology business.industry Brief Report interleukin-6 erdheim–chester disease Neoplasms. Tumors. Oncology. Including cancer and carcinogens Magnetic resonance imaging RC581-607 medicine.disease inflammation histiocytosis Histiocytosis 030104 developmental biology Oncology chemistry 030220 oncology & carcinogenesis biology.protein Immunologic diseases. Allergy business |
| Zdroj: | OncoImmunology, Vol 6, Iss 6 (2017) |
| DOI: | 10.1080/2162402X.2017.1318237 |
| Popis: | Treatment of ErdheimâChester disease (ECD), a rare non-Langerhans histiocytosis, relies on interferon-α, 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-α. 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. |
| Databáze: | OpenAIRE |
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