Behçet's disease associated with superior vena cava syndrome without thrombosis

Clinical Rheumatology
Tadeu Ferreira de PaivaBranca Dias Batista de Souza

Abstract

Behçet's disease is a multisystemic vasculitis of unknown etiology, which is characterized by recurrent urogenital ulceration, cutaneous eruptions, ocular manifestations, arthritis and vasculitis, and its diagnosis is based on clinical criteria. Superior vena cava (SVC) thrombosis is a rare but well-recognized manifestation of Behçet's disease, whereas SVC syndrome due to vasculopathy, without evidence of thrombosis, has not yet been described in the literature. The authors report the case of a patient with Behçet's disease, who presented SVC syndrome with reduction in the lumen of the SVC due to thickening of the vessel wall, without evidence of thrombosis upon computed tomography and magnetic angioresonance. The patient received early anticoagulant therapy, corticosteroid and monthly cyclophosphamide pulse therapy. Clinical control without recurrence was observed after 6 months of follow-up. Behçet's disease should be suspected in young patients presenting with SVC syndrome, in the absence of thrombosis or of a hypercoagulable state.

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Citations

Jun 21, 2008·The British Journal of Dermatology·S-H OhD Bang
Dec 3, 2010·Current Opinion in Rheumatology·Kenneth T CalamiaMelike Melikoglu
Jun 23, 2020·Canadian Respiratory Journal : Journal of the Canadian Thoracic Society·Kemal ÖdevCelalettin Korkmaz

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