Superior vena cava syndrome without thrombosis revealing Behçet's disease: Two cases

Superior vena cava (SVC) thrombosis is a rare but well-recognized manifestation of Behcet's disease, whereas SVC syndrome due to vasculopathy, without evidence of thrombosis, has only been described a single time in the literature. We report two cases of this exceptional association. The two patients presented with swelling of the face and oral aphtosis, the SVC stenosis was evidenced by angio-CT and classical cavography. In each case, a combination of anticoagulant and immunosuppressive (corticosteroids and azathioprine) treatments allowed a significant improvement of SVC diameter. In one of the patients, an angioplasty of SVC was also performed. We believe there is a continuum between non-obstructive and obstructive vasculopathy (thrombosis) of SVC. Inflammatory vascular injury secondary to vasculitis is considered to be the primary reason for thrombosis in Behcet's disease. Thickening of the venous wall observed in our patients probably reflects this inflammatory vascular injury. In conclusion, Behcet's disease should be considered in the differential diagnosis of SVC syndrome not related to thrombosis.