Session Title: Vasculitis III
Session Type: Abstract Submissions (ACR)
Background/Purpose: Neurological involvement occurs in 5.3 to up to 59% of patients with Behçet’s disease (BD). Although the clinical and imaging features of neuro-Behçet’s disease (NBD) have been extensively described, few studies have reported on the long term outcome, treatment and prognosis of NBD by multivariate analyses. Moreover, no large study has focused on the impact of immunosuppressants on the outcome of NBD. In this study, we sought to report the long-term outcome of neurological involvement in patients with Behçet’s disease (BD).
Methods: Retrospective analysis of 115 patients fulfilling the International Criteria for BD [57% male with median [Q1-Q3] age of 37 [30-46] years] with neuro-Behçet’s disease (NBD) after exclusion of cerebral venous thrombosis. Factors associated with relapses of NBD, dependence and mortality were assessed by multivariate analysis. The event-free survival was calculated using Kaplan-Meier curves and a multivariate Cox proportional hazard ratio model was performed.
Results: Seventy eight (68%) patients presented with an acute onset of NBD and 37 (32%) with a progressive course. The HLA B51 allele was carried by 49% of patients. Overall, 46/115 (40%) patients had a severe initial disability status, represented by a Rankin score ≥ 3. The 5 and 7 years event-free survival rate were of 65% and 53%, respectively. In multivariate analysis, HLA-B51 positivity was independently associated with the risk of relapses of NBD (OR=4.2 [1.6-10.9]). After a median follow-up of 73 [59-102] months, 21 (25.2%) patients became dependent or died. Factors independently associated with poor outcome were a paresis at onset (OR=6.47 [1.73-24.23]) and a brainstem location of inflammatory lesions on MRI (OR=8.41 [1.03-68.43]). All the 115 patients were treated with glucocorticosteroids, including 53/115 (46.1%) with cyclophosphamide and 40/115 (34.8%) with azathioprine. A trend towards a longer event-free survival was observed in severe NBD patients (i.e. Rankin score ≥ 3 at onset) receiving intravenous cyclophosphamide compared with those treated with azathioprine (p =0.06).
Conclusion: NBD is a severe condition in which HLA-B51 carriers seems to have a worse prognosis.
J. C. Piette,
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