Background Central nervous system (CNS) involvement in Behcet’s disease (BD) is one of its most serious complications (neuro-Behcet’s disease [NBD]). It is unclear and still under debate whether cyclosporine-induced neurological manifestations might be different from acute neurological events of NBD.
Objectives The current studies therefore compared the clinical characteristics of cyclosporine-related acute neurological events with cyclosporine-unrelated acute manifestations of NBD.
Methods A multicenter retrospective survey was performed on BD patients who fulfilled the diagnostic criteria of the international study group and presented neurological manifestations between 1987 and 2008. The diagnosis of NBD, including cyclosporine-related and cyclosporine-unrelated acute neurological events, was confirmed by retrospective review of the clinical records. Kaplan-Meier analysis was performed to compare the relapse ratio.
Results A total of 144 BD patients were studied, including 76 with NBD, who showed acute neurological events. Twenty-six of the 76 patients had been taking cyclosporin at the onset of the acute neurological events. All the 26 patients with cyclosporin and 43 of the 50 patients without cyclosporin had eye involvement. There were no significant differences in demographic features, clinical symptoms, MRI findings, laboratory findings (serum CRP, cerebrospinal fluid cell counts and IL-6) between patients with cyclosporin and those without cyclosporin. On analysis of 66 acute neurological events (21 cyclosporin-related, 45 cyclosporin-unrelated), there were no significant differences in the need for steroid therapy including pulse therapy and in the responses to the treatment. Of note, patients with cyclosporin -unrelated events showed significantly higher relapse-ratio than those with cyclosporine-related events (HR 8.452, 95% CI: 1.330-10.89, p=0.0127).
Conclusions These results therefore indicate that cyclosporin-related acute neurological manifestations are almost identical with cyclosporin-unrelated acute events of NBD, except for the paucity of relapse on withdrawal of cyclosporin. The data also suggest that cyclosporin should be regarded as one of the risk factors for NBD.
Disclosure of Interest None Declared
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