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Anakinra treatment in drug-resistant Behcet’s disease: a case series

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Abstract

The study objective was to report treatment with an interleukin (IL)-1 receptor antagonist, anakinra, in patients with multiorgan Behcet's disease (BD). Comparison of clinical manifestations, previous treatments, markers of inflammation, concomitant medications, treatment regimen modifications, relapses, and adverse events before and during anakinra administration among patients with BD were evaluated. Nine BD patients (mean age 34.55 ± 16.30 years) refractory to tumor necrosis factor blockers and standardized therapies are reported in our survey. Their mean age at disease onset was 25 ± 13.88 years and their overall disease duration was 9.55 ± 5.33 years. All patients were positive for the HLA-B51 allele. Within 1 or 2 weeks following the initiation of anakinra, eight out of nine patients promptly responded, and most of them were maintained on 100 mg of daily anakinra with low doses of prednisone. However, most patients experienced a relapse in one or more clinical manifestations over time (mean time to relapse 29 ± 21.65 weeks), and only one patient remained completely under control on anakinra monotherapy. Despite a relapse in one or more disease manifestations, treatment was continued in most patients for a mean period of 13.75 ± 6.49 months. No serious adverse events occurred. Eight out of nine refractory BD patients showed a prompt improvement after starting anakinra, supporting the concept that IL-1 plays a pathological role in this disease. Nevertheless, after several months, most patients experienced a relapse. It remains unclear whether increasing the dose of anakinra would have prevented the reoccurrence of disease activity.

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Authors and Affiliations

  1. Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy

    Luca Cantarini, Antonio Vitale, Orso Maria Lucherini, Bruno Frediani, Ilaria Bertoldi & Mauro Galeazzi

  2. Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Viale Bracci 1, 53100, Siena, Italy

    Luca Cantarini

  3. Department of Pediatrics, Rheumatology Unit, Anna Meyer Children’s Hospital, University of Florence, Florence, Italy

    Perla Scalini, Gabriele Simonini & Rolando Cimaz

  4. Department of Medicine, University of Colorado Denver, Aurora, CO, USA

    Charles A. Dinarello

  5. Department of Medicine, University Medical Center Nijmegen, Nijmegen, The Netherlands

    Charles A. Dinarello

  6. Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy

    Donato Rigante

  7. Ophthalmology and Neurosurgery Department, University of Siena, Siena, Italy

    Rossella Franceschini & Giulia Borsari

  8. Rheumatology Unit, Department of Medicine, University of Padova, Padua, Italy

    Francesco Caso & Leonardo Punzi

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  1. Luca Cantarini
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  2. Antonio Vitale
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  3. Perla Scalini
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  4. Charles A. Dinarello
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  10. Orso Maria Lucherini
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Correspondence to Luca Cantarini.

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Luca Cantarini and Antonio Vitale contributed equally.

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Cantarini, L., Vitale, A., Scalini, P. et al. Anakinra treatment in drug-resistant Behcet’s disease: a case series. Clin Rheumatol 34, 1293–1301 (2015). https://doi.org/10.1007/s10067-013-2443-8

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  • DOI: https://doi.org/10.1007/s10067-013-2443-8

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