Clinical features and management of venous thromboembolism in patients with Behçet’s syndrome: a single-center case–control study

Abstract

Almost one third of patients with Behçet's syndrome (BS) display vascular involvement. However, data regarding the prevalence and management of venous thromboembolism (VTE) in BS are scanty. We assessed the differential characteristics between patients with and without VTE and the factors associated with VTE incidence. A case–control study in a cohort of patients with BS was performed. 57 patients were included (56.1% women) with a mean follow-up of 10.56 (± 10.7) years. Mean age at diagnosis of BS and diagnosis of the first VTE episode was 34.7 (± 12.1) and 31.2 (± 8.9) years, respectively. Erythema nodosum (OR 4.6, CI 95% 1.2–18.1) and fever (OR 8.2, CI 95% 1.6–42.1) were associated with a higher risk of VTE. 26 episodes of VTE were registered in 12/57 (21%) patients. 83.3% of patients were not diagnosed with BS when the first episode of VTE occurred and, among them, the episode of VTE led to the diagnosis of BS in 40% of cases. Half of patients had at least one VTE recurrence. The absence of immunosuppressive treatment was associated with a higher risk of developing a first episode of VTE (OR 20 CI 95% 19.2–166.6). All patients were treated with anticoagulation and 75% were treated with immunosuppressants after the first VTE event. The diagnosis of VTE usually precedes that of BS, with a high frequency of VTE recurrence. Erythema nodosum and fever were associated with a higher risk of VTE, while the immunosuppressants showed a protective role for the development of VTE.

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Abbreviations

BS:

Behçet’s syndrome

VTE:

Venous thromboembolism

ISGBD:

International Study Group for Behçet’s Disease

ITR-ICBD:

International Team for the Revision of the International Criteria for Behçet's Disease

DVTLE:

Deep venous thrombosis of lower extremities

SVT:

Superficial venous thrombosis

ICV:

Inferior cava vein

CECT:

Contrast-enhanced computed tomography (CECT)

CTPA:

Computed tomography pulmonary angiography (CTPA)

HLA:

Human leukocyte antigen

ICBD:

International criteria for BD

LEVT:

Lower extremities venous thrombosis

DVT:

Deep venous thrombosis

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Funding

This research did not receive any specific Grant from funding agencies in the public, commercial, or not-for-profit sectors.

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Contributions

Conception and design: NT-S, FG-V. Administrative support: NT-S, FG-V. Provision of study materials or patients: NT-S, FG-V. Collection and assembly of data: NT-S, FG-V. Data analysis and interpretation: NT-S, FG-V, PD-R. Manuscript writing: NT-S, FG-V. Final approval of manuscript: all authors.

Corresponding author

Correspondence to Francisco Galeano-Valle.

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Statement of human and animal rights

This study was carried out following the international ethical recommendations for conducting research in humans in the latest revision of the Declaration of Helsinki, as well as those established in the Good Clinical Practice Guidelines and in the current legislation. The study was approved by the Institutional Ethics Committee (03/2019).

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Informed consent was not requested because it was a retrospective study.

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Toledo-Samaniego, N., Galeano-Valle, F., Pinilla-Llorente, B. et al. Clinical features and management of venous thromboembolism in patients with Behçet’s syndrome: a single-center case–control study. Intern Emerg Med 15, 635–644 (2020). https://doi.org/10.1007/s11739-019-02237-7

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Keywords

  • Behçet’s syndrome
  • Immunosuppressive agents
  • Corticosteroids
  • Embolism