A Rare Case of Porto-Sinusoidal Vascular Disease Associated with Systemic Lupus Erythematosus and Antiphospholipid Syndrome.

Abstract

Porto-sinusoidal vascular disease is a medical condition characterized by increased blood pressure in the portal vein in the absence of cirrhosis. This condition is considered poorly defined because its exact cause is unknown. It has been reported to be associated with several autoimmune diseases, including systemic lupus erythematosus and antiphospholipid syndrome. We report a case of 49-year-old Indian lady with a background history of systemic lupus erythematosus with secondary antiphospholipid syndrome who was found to have esophageal varices from an esophagogastroduodenoscopy performed for iron deficiency anemia. A follow-up ultrasound and computed tomography of the abdomen confirmed the absence of cirrhosis and excluded portal vein thrombosis as a potential etiology for portal hypertension leading to the diagnosis of porto-sinusoidal vascular disease. She started on propranolol as primary prophylaxis for variceal bleeding. The patient remained well during subsequent follow-ups with the absence of new or bleeding varices during annual esophagogastroduodenoscopy surveillance. This case contributes to the further understanding of PVSD in SLE and secondary antiphospholipid syndrome. Further research is needed to enhance our understanding of its pathophysiology and to help formulate comprehensive guidelines for the effective management of this condition.