The abdominal manifestations of the antiphospholipid syndrome

Objectives. To study the abdominal manifestations of the antiphospholipid syndrome (APS). Methods. We reviewed the medical literature from 1968 to 2006 using MEDLINE and the key words: APS, anticardiolipin antibodies, lupus anticoagulant, antiphospholipid (aPL) antibodies, catastrophic antiphospholipid syndrome, liver, hepatic biliary, pancreas, spleen, gastrointestinal and abdominal. Results. Liver involvement is the most frequent abdominal manifestation associated with APS. Various hepatic manifestations have been reported including Budd-Chiari syndrome, hepatic-veno-occlusive disease and occlusion of small hepatic veins, nodular regenerative hyperplasia, hepatic infarction, cirrhosis, portal hypertension, autoimmune hepatitis and biliary cirrhosis. Acute intestinal infarction, intestinal angina, and intestinal bleeding have also been reported in association with aPL in addition to few sporadic cases of splenic infarction and acute pancreatitis. Conclusion. A high index of suspicion for any signs of abdominal involvement should be considered in patients with APS. In addition screening for aPL should be carried out in patients who present with hepatic vein occlusion and unexplained signs of intestinal angina.