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

Lupus. 2007;16(1):56-8. doi: 10.1177/0961203306072390.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • 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*

Substances

  • Methylenetetrahydrofolate Reductase (NADPH2)