"Downhill" esophageal varices and occlusion of superior and inferior vena cavas due to a systemic venulitis.

A patient with a 10-year history of episodes of venous thrombosis, a high ESR, an elevated plasma polyclonal 1gG 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.