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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International Study Group for Behçet’s Disease
International Team for the Revision of the International Criteria for Behçet's Disease
Deep venous thrombosis of lower extremities
Superficial venous thrombosis
Inferior cava vein
Contrast-enhanced computed tomography (CECT)
Computed tomography pulmonary angiography (CTPA)
Human leukocyte antigen
International criteria for BD
Lower extremities venous thrombosis
Deep venous thrombosis
Yazici H, Seyahi E, Hatemi G, Yazici Y (2018) Behçet syndrome: a contemporary view. Nat Rev Rheumatol 14:107–119 (Erratum in Nat Rev Rheumatol. 2018 14:119)
Seyahi E (2016) Behçet’s disease: how to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol [Internet] 30(2):279–295
Emmi G, Silvestri E, Squatrito D, Prisco D, Emmi L (2014) Behçet’s syndrome pathophysiology and potential therapeutic targets. Intern Emerg Med 9:257–265
Seyahi E (2019) Phenotypes in Behçet’s syndrome. Intern Emerg Med 14(5):677–689
Emmi G, Bettiol A, Silvestri E, Di G, Matteo S, Claudia B et al (2018) Vascular Behçet’s syndrome: an update. Intern Emerg Med 14(5):645–652
Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V et al (2003) The long-term mortality and morbidity of Behçet syndrome a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 82:60–76
Fabiani C, Vitale A, Orlando I, Sota J, Capozzoli M, Franceschini R et al (2017) Quality of life impairment in Behçet’s disease and realationship with disease activity: a prospective study. Intern Emerg Med 12:947–955
Zeidan MJ, Saadoun D, Garrido M, Klatzmann D, Six A, Cacoub P (2016) Behçet's disease physiopathology: a contemporary review. Auto Immun Highlights 7:4
Emmi G, Becatti M, Bettiol A, Hatemi G, Prisco D, Fiorillo C (2019) Behçet’s syndrome as a model of thrombo-inflammation: the role of neutrophils. Front Immunol 10:1085
Becatti M, Emmi G, Silvestri E, Bruschi G, Ciucciarelli L, Squatrito D et al (2016) Neutrophil activation promotes fibrinogen oxidation and thrombus formation in Behçet disease. Circulation 133:302–311
Becatti M, Emmi G, Bettiol A, Silvestri E, Scala G, Taddei N et al (2019) Behçet's syndrome as a tool to dissect the mechanisms of thrombo-inflammation: clinical and pathogenetic aspects. Clin Exp Imunol. 195(3):322–333
Mantovani A, Cassatella MA, Constatini C, Jaillon S (2011) Neutrophils in the activation and regulation of innate and adaptive immunity. Nat Rev Inmunol 11:519–531
Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F et al (2018) 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis 77(6):808–818
Fei Y, Li X, Lin S, Song X, Wu O, Zhu Y et al (2013) Major vascular involvement in Behçet's disease: a retrospective study of 796 patients. Clin Rheumatol 32:845–852
Seyahi E, Yazici H (2015) Behçet’s syndrome : pulmonary vascular disease. Curr Opin Rheumatol 27:18–23
International Study Group for Behçet’s Disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1078–1080
Davatchi F, Assaad-Khalil S, Calamia KT, Crook JE, Sadeghi-Abdollahi B, Schirmer M et al (2014) International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 28:338–347
Rodríguez-Carballeira M, Solans R, Larrañaga JR, García-Hernández FJ, Ríos-Fernández R, Nieto J et al (2018) Venous thrombosis and relapses in patients with Behçet’s disease. Descriptive analysis from Spanish network of Behçet’s disease (REGEB cohort). Autoimmune Diseases Study Group (GEAS). Clin Exp Rheumatol 36((6 Suppl 115)):40–44
Alibaz-Oner F, Karadeniz A, Yılmaz S, Balkarlı A, Kimyon G, Yazıcı A et al (2015) Behçet disease with vascular involvement: effects of different therapeutic regimens on the incidence of new relapses. Med Baltim 94:e494
Desbois AC, Wechsler B, Resche-Rigon M, Piette JC, Huong Dle T, Amoura Z et al (2012) Immunosuppressants reduce venous thrombosis relapse in Behçet's disease. Arthritis Rheum 64:2753–2760
Wu X, Li G, Huang X, Wang L, Liu W, Zhao Y et al (2014) Behçet’s disease complicated with thrombosis a report of 93 Chinese cases. Medicine (Baltimore) 93(28):e263
Tohmé A, Aoun N, El-Rassi B, Ghayad E (2003) Vascular manifestations of Behçet’s disease. Eighteen cases among 140 patients. Joint Bone Spine 70:384–389
Tascilar K, Melikoglu M, Ugurlu S, Sut N, Caglar E, Yazici H et al (2018e) Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course. Rheumatol Oxf 53:2018e22
Galeano-Valle F, Demelo-Rodriguez P, Álvarez-Sala-Walther L, Pinilla-Llorente B, Echenagusia-Boyra MJ, Rodriguez-Abella H et al (2018) Intracardiac thrombosis in Behçet’s Disease successfully treated with immunosuppressive agents: a case of vascular pathergy phenomenon. Intractable Rare Dis Res 7(1):54–57
Oblitas CM, Galeano-Valle F, Toledo-Samaniego N, Pinilla-Llorente B, Del Toro-Cervera J, Álvarez-Luque A et al (2019) Budd-Chiari syndrome in Behçet's disease successfully managed with immunosuppressive and anticoagulant therapy: a case report and literature review. Intractable Rare Dis Res 8(1):60–66
Ahn JK, Lee YS, Jeon CH, Koh EM, Cha HS (2008) Treatment of venous thrombosis associated with Behcet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clin Rheumatol 27:201–205
Tayer-Shifman OE, Seyahi E, Nowatzky J, Ben-Chetrit E (2012) Major vessel thrombosis in Behçet’s disease: the dilemma of anticoagulant therapy—the approach of rheumatologists from different countries. Clin Exp Rheumatol 30:735–740
Hamuryudan V, Er T, Seyahi E, Akman C, Tüzün H, Fresko I et al (2004) Pulmonary artery aneurysm in Behçet syndrome. Am J Medd 117:867–870
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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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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
- Behçet’s syndrome
- Immunosuppressive agents