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

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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.

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Acknowledgements

The data underlying this article will be shared at reasonable request to the corresponding author.

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.

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Authors and Affiliations

  1. Department of Internal Medicine, Division of Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey

    Didem Sahin Eroglu, Murat Torgutalp, Serdar Sezer, Mucteba Enes Yayla & Askin Ates

  2. Department of Neurology, Ankara University Faculty of Medicine, Ankara, Turkey

    Canan Yucesan

  3. Department of Dermatology, Ankara University Faculty of Medicine, Ankara, Turkey

    Ayse Boyvat

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  1. Didem Sahin Eroglu
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  2. Murat Torgutalp
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Correspondence to Didem Sahin Eroglu.

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Ethical approval

We obtained approval from the ethics committee of Ankara University (Approval number I6-364-20). Our study did not require consent due to the retrospective design of the study, in which ethical approval is adequate. We conducted our research in accordance with the Declaration of Helsinki.

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Sahin Eroglu, D., Torgutalp, M., Yucesan, C. et al. Prognostic factors for relapse and poor outcome in neuro-Behçet’s syndrome: results from a clinical long-term follow-up of a single centre. J Neurol 269, 2046–2054 (2022). https://doi.org/10.1007/s00415-021-10787-0

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