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Cerebral venous sinus thrombosis in Behçet’s disease: a retrospective case-control study

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Abstract

The objective of the study is to investigate the clinical features of Behçet’s disease (BD) complicated with cerebral venous sinus thrombosis (CVST). We retrospectively analyzed the clinical features of age- and gender-matched BD patients with and without CVST. Of the 840 BD patients enrolled, 21 (2.5%, 11 female, mean age 30.3 ± 9.5) were diagnosed as CVST. Eighteen patients (85.7%) developed neurologic manifestations after BD onset, with an average interval of 47.6 months (SD 36.1). Onset of neurologic symptoms was mostly progressive (89.5%), with headache (95.2%) being the most common symptom. Intracranial hypertension occurred in 17 cases (89.5%). Thrombosis was detected mostly in transverse (71.4%) and superior sagittal (66.7%) sinuses, with more frequently (66.7%) dual or multiple sinuses involved. In addition, extra cranial thrombosis was presented in 11 cases. Cerebral infarction was presented in four cases. Compared with BD patients without CVST, the prevalence of extra cranial vascular involvement (52.4 versus 20.6%; P = 0.005) and BDCAF 2006 score (3.7 ± 1.4 versus 1.8 ± 1.3; P = 1.7863 × 10−7) was higher in BD patients with CVST. During a median 7-month follow-up (range 0.3–88.8 months), 18 patients (85.7%) achieved remission following glucocorticoid, immunosuppressant therapy, anticoagulants, and dehydration, although 3 patients experienced (14.3%) flare. No death was reported. CVST is rare complication of BD and predominately progressively developed in active BD and after BD onset. It typically features intracranial hypertension syndrome and is frequently complicated with extra cranial thrombosis; thus, systemic thrombus screening is highly recommended. Promptly, diagnosis and aggressive treatment are keys to improve the prognosis.

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Author notes

  1. Jing Shi and Xinxiang Huang contributed equally to this work.

Authors and Affiliations

  1. Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

    Jing Shi, Li Wang, Jinjing Liu, Xiaofeng Zeng & Wenjie Zheng

  2. Department of Hematology and Rheumatology, People’s Hospital of Guangxi Zhuang Autonomous Region, Nanning, China

    Xinxiang Huang

  3. Department of Nephrology, Inner Mongolia Hospital of Traditional Chinese Medicine, Hohhot, China

    Guohua Li

  4. Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

    Yan Xu

Authors
  1. Jing Shi
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  2. Xinxiang Huang
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  3. Guohua Li
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  4. Li Wang
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  6. Yan Xu
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  8. Wenjie Zheng
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Contributions

All authors made substantial contributions to the conception and design of this study. Xinxiang Huang, Guohua Li, and Jing Shi acquired the data. Jing Shi performed the data analysis and interpretation and wrote the manuscript. Wenjie Zheng provided critical revisions to the manuscript. Li Wang, Jinjing Liu, Yan Xu, and Xiaofeng Zeng also critically reviewed the manuscript and provided valuable input. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Wenjie Zheng.

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Ethical standards

We received a waiver of ethics approval from the Institutional Review Board at our institution because this was a retrospective and observational study. Because the study was based on a review of medical records that had been obtained for clinical purposes, written informed consent was waived. The patient’s records/information was anonymized and deidentified prior to analysis.

Funding

This study was supported by the National Natural Science Foundation of China Grants (81571598), National Key Research and Development Program: “Precise Medical Research” (2016YFC0906201), and CAMS Initiative for Innovative Medicine (2016-I2M-1-013).

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Shi, J., Huang, X., Li, G. et al. Cerebral venous sinus thrombosis in Behçet’s disease: a retrospective case-control study. Clin Rheumatol 37, 51–57 (2018). https://doi.org/10.1007/s10067-017-3718-2

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  • DOI: https://doi.org/10.1007/s10067-017-3718-2

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