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.