title: Anakinra treatment in drug-resistant Behcet's disease: a case series.
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.
authors.affiliation: Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy, cantariniluca@hotmail.com.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.forename: Antonio
authors.initials: A
authors.lastname: Vitale
authors.forename: Perla
authors.initials: P
authors.lastname: Scalini
authors.forename: Charles A
authors.initials: CA
authors.lastname: Dinarello
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
authors.forename: Rossella
authors.initials: R
authors.lastname: Franceschini
authors.forename: Gabriele
authors.initials: G
authors.lastname: Simonini
authors.forename: Giulia
authors.initials: G
authors.lastname: Borsari
authors.forename: Francesco
authors.initials: F
authors.lastname: Caso
authors.forename: Orso Maria
authors.initials: OM
authors.lastname: Lucherini
authors.forename: Bruno
authors.initials: B
authors.lastname: Frediani
authors.forename: Ilaria
authors.initials: I
authors.lastname: Bertoldi
authors.forename: Leonardo
authors.initials: L
authors.lastname: Punzi
authors.forename: Mauro
authors.initials: M
authors.lastname: Galeazzi
authors.forename: Rolando
authors.initials: R
authors.lastname: Cimaz
mesh_terms: D000328:Adult; D000483:Alleles; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001528:Behcet Syndrome / Q000188:drug therapy*; D015415:Biomarkers / Q000097:blood; D002648:Child; D004351:Drug Resistance; D005260:Female; D059949:HLA-B51 Antigen / Q000235:genetics; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D016896:Treatment Outcome; D014605:Uveitis / Q000188:drug therapy; D055815:Young Adult
chemical_list: D018501:Antirheumatic Agents; D015415:Biomarkers; D059949:HLA-B51 Antigen; D053590:Interleukin 1 Receptor Antagonist Protein