Prognostic factors for relapse and poor outcome in neuro-Behçet’s syndrome: results from a clinical long-term follow-up of a single centre

Objective To describe the clinical characteristics of neuro-Behçet’s syndrome (NBS) and to define the factors associated with relapses and poor outcome. Methods Among 2118 patients with Behçet’s syndrome who fulfilled the international study group criteria , 208 (9.8%) patients had NBS. Retrospective data of 125 NBS patients (55.5% male; mean age 37.2 ± 11.8 years) were analysed. We divided patients into two subgroups, either parenchymal (p-NBS) or non-parenchymal (np-NBS), according to international consensus recommendations for NBS. We assessed the predictor factors associated with relapse and poor outcome—which was defined as a modified Rankin score (mRS) ≥ 3 at last follow-up and/or death—using Cox and logistic regression analyses, respectively. Results In total, 79 (63.2%) patients presented with p-NBS and 46 (36.8%) presented with np-NBS. Ocular involvement was more common in p-NBS than np-NBS (55.7% vs. 37.0%, p = 0.04), whereas vascular involvement excluding cerebral vein thrombosis was more frequent in patients with np-NBS (19.0% vs. 52.2%, p < 0.001). Forty-two patients (33.6%) experienced at least one relapse. Factors associated with relapse were BS diagnosis at a younger age and cranial nerve dysfunction (HR 0.96 95% CI 0.93–0.99 and 2.36 95% CI 1.23–4.52, respectively). After a median of 68 (Q1–Q3: 25–125) months, 23 patients (18.4%) had a poor outcome. Indicators of a poor outcome were higher initial mRS and the progressive p-NBS type (OR 8.28 95% CI 1.04–66.20 and 33.57 95% CI 5.99–188.21, respectively). Conclusion Our findings indicate that clinical characteristics and prognosis differ between NBS subgroups, of which patients with p-NBS have worse outcomes.