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Erschienen in:

19.02.2022

Arterial and venous involvement in Behçet’s syndrome: a narrative review

verfasst von: Neera Toledo-Samaniego, Crhistian Mario Oblitas, Eduardo Peñaloza-Martínez, Jorge del-Toro-Cervera, Luis Antonio Alvarez-Sala-Walther, Pablo Demelo-Rodríguez, Francisco Galeano-Valle

Erschienen in: Journal of Thrombosis and Thrombolysis | Ausgabe 1/2022

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Abstract

Behçet syndrome (BS) is a unique type of vasculitis that affects veins and arteries of all sizes, leading to recurrent vascular events, mostly venous thrombosis. The prevalence of venous thromboembolism in BS patients ranges between 15 and 40%. Thrombosis is usually an early manifestation leading to diagnosis of BS in up to 40% of patients. BS is per se a model of inflammation-induced thrombosis. The primary autoimmune response activates lymphocytes that in turn produce a cytokine cascade that activates neutrophils, which modify the secondary structure of fibrinogen making it less susceptible to plasmin-induced lysis. This leads to endothelial dysfunction, platelet activation and overexpression of tissue factor leading to inflammatory thrombi, usually attached to the wall. The pathogenesis of thrombosis is especially relevant to direct the specific treatment, that is based on immunosuppression rather than anticoagulation. Superficial vein thrombosis (SVT) and deep vein thrombosis (DVT) are the most common form of thrombosis in BS, but thrombosis in atypical sites (cava vein, suprahepatic veins, intracardiac thrombus) and arterial involvement can also occur. We assessed the latest update of the European League Against Rheumatism recommendations for the management of BS. Vascular Behçet treatment is usually based of immunosuppressants, and the role of anticoagulation remains controversial. The use of interventional and surgical procedures should be carefully evaluated, due to the risk of triggering a vascular pathergy phenomenon.

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Titel: Arterial and venous involvement in Behçet’s syndrome: a narrative review
verfasst von: Neera Toledo-Samaniego
Crhistian Mario Oblitas
Eduardo Peñaloza-Martínez
Jorge del-Toro-Cervera
Luis Antonio Alvarez-Sala-Walther
Pablo Demelo-Rodríguez
Francisco Galeano-Valle
Publikationsdatum: 19.02.2022
Verlag: Springer US
Erschienen in: Journal of Thrombosis and Thrombolysis / Ausgabe 1/2022
Print ISSN: 0929-5305
Elektronische ISSN: 1573-742X
DOI: https://doi.org/10.1007/s11239-022-02637-1

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