Spontaneous splenorenal shunt in a patient with antiphospholipid syndrome: the first case reported

• Published in: Lupus Vol. 16; no. 1; pp. 56 - 58
• Main Authors: Cacciapaglia, F, Vadacca, M, Coppolino, G, Buzzulini, F, Rigon, A, Zennaro, D, Zardi, E, Afeltra, A
• Format: Journal Article
• Language: English
• Published: Thousand Oaks, CA SAGE Publications 01-01-2007; Sage Publications Ltd
• Subjects: Antiphospholipid Syndrome - complications; Female; Fistula - diagnostic imaging; Fistula - etiology; Genetic Predisposition to Disease; Heterozygote; Humans; Mesenteric Vascular Occlusion - etiology; Methylenetetrahydrofolate Reductase (NADPH2) - deficiency; Methylenetetrahydrofolate Reductase (NADPH2) - genetics; Middle Aged; Portal Vein - pathology; Protein C Deficiency - complications; Protein S Deficiency - complications; Renal Veins - diagnostic imaging; Renal Veins - pathology; Splenic Vein - diagnostic imaging; Splenic Vein - pathology; Thrombophilia - etiology; Thrombophilia - genetics; Ultrasonography, Doppler, Color; Venous Thrombosis - etiology; Color-Doppler sonography; total portal thrombosis; primary antiphospholipid syndrome; spontaneous splenorenal shunt
• ISSN: 0961-2033; 1477-0962
• DOI: 10.1177/0961203306072390

The antiphospholipid syndrome (APS) is an autoimmune disorder, characterized by a wide spectrum of clinical manifestations. Thromboembolic events, with a greater involvement of extremities veins, are the most common features, and obstruction of abdominal vessels are sporadically reported. We present a singular case of a patient with primary APS (PAPS) that developed a spontaneous splenorenal shunt, secondary to a total portal, mesenteric and splenic vein thrombosis. Spontaneous splenorenal shunt, an uncommon circumstance reported in cirrhotic disease, to the best of our knowledge, has not been previously described in PAPS.