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

Abstract

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.

Keywords: Behçet’s syndrome; Neuro-Behçet’s syndrome; Neurological involvement; Poor outcome; Prognosis; Relapse.