“Downhill” Esophageal Varices and Occlusion of Superior and Inferior Vena Cavas Due to a Systemic Venulitis

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A patient with a 10-year history of episodes of venous thrombosis, a high ESR, an elevated plasma polyclonal lgG concentration, and bleeding from esophageal varices had obstruction of both superior and inferior vena cavas with “downhill” esophageal varices and a normal portal vein with normal portal pressure. Extensive investigations revealed no predisposing factor for the venous thromboses, but the patient made a good clinical response to steroids and dapsone, with no further episodes of bleeding nor evidence of major venous thrombosis. The causes and outcome of “downhill” varices are discussed.

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