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Clinical Rheumatology

, Volume 34, Issue 7, pp 1293–1301 | Cite as

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

  • Luca Cantarini
  • Antonio Vitale
  • Perla Scalini
  • Charles A. Dinarello
  • Donato Rigante
  • Rossella Franceschini
  • Gabriele Simonini
  • Giulia Borsari
  • Francesco Caso
  • Orso Maria Lucherini
  • Bruno Frediani
  • Ilaria Bertoldi
  • Leonardo Punzi
  • Mauro Galeazzi
  • Rolando Cimaz
Case Based Review

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.

Keywords

Behcet's disease Biologic agents Interleukin-1 inhibition Uveitis 

Notes

Disclosures

None

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Copyright information

© Clinical Rheumatology 2013

Authors and Affiliations

  • Luca Cantarini
    • 1
    • 2
  • Antonio Vitale
    • 1
  • Perla Scalini
    • 3
  • Charles A. Dinarello
    • 4
    • 5
  • Donato Rigante
    • 6
  • Rossella Franceschini
    • 7
  • Gabriele Simonini
    • 3
  • Giulia Borsari
    • 7
  • Francesco Caso
    • 8
  • Orso Maria Lucherini
    • 1
  • Bruno Frediani
    • 1
  • Ilaria Bertoldi
    • 1
  • Leonardo Punzi
    • 8
  • Mauro Galeazzi
    • 1
  • Rolando Cimaz
    • 3
  1. 1.Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le ScotteUniversity of SienaSienaItaly
  2. 2.Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, Policlinico Le ScotteUniversity of SienaSienaItaly
  3. 3.Department of Pediatrics, Rheumatology Unit, Anna Meyer Children’s HospitalUniversity of FlorenceFlorenceItaly
  4. 4.Department of MedicineUniversity of Colorado DenverAuroraUSA
  5. 5.Department of MedicineUniversity Medical Center NijmegenNijmegenThe Netherlands
  6. 6.Institute of PediatricsUniversità Cattolica Sacro CuoreRomeItaly
  7. 7.Ophthalmology and Neurosurgery DepartmentUniversity of SienaSienaItaly
  8. 8.Rheumatology Unit, Department of MedicineUniversity of PadovaPaduaItaly

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