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Rheumatology Advance Access originally published online on July 17, 2007
Rheumatology 2007 46(11):1641-1647; doi:10.1093/rheumatology/kem158
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© The Author 2007. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

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The abdominal manifestations of the antiphospholipid syndrome

I. Uthman and M. Khamashta1

Division of Rheumatology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon and 1Lupus Research Unit, The Rayne Institute, Guy's, King's and St Thomas’ School of Medicine, St. Thomas’ Hospital, London, UK

Correspondence to: Imad W. Uthman, MD, MPH, American University of Beirut, Medical Center, PO Box: 113-6044, Beirut, Lebanon. E-mail: iuthman{at}aub.edu.lb


  Abstract

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.

KEY WORDS: Anticardiolipin antibodies, Primary biliary cirrhosis, Primary sclerosing cholangitis, Antiphospholipid antibodies, Antiphospholipid syndrome, Autoimmune hepatitis

Submitted 1 March 2007; revised version accepted 18 May 2007.
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