Purpose: The purpose of this work was to evaluate the CT features of the abdominal manifestations of primary antiphospholipid syndrome (PAPS).
Method: Of the 32 patients who were confirmed to have PAPS among 751 patients with elevated antiphospholipid antibodies during a 2 year period, we retrospectively reviewed the 14 patients who underwent abdominal CT. The clinical indications for abdominal CT included abdominal pain, abdominal distension, or lower leg swelling. CT findings were analyzed with regard to the abdominal vascular system and abdominal organ involvement patterns as well as ancillary findings.
Results: Of the 14 patients with PAPS, 10 had involvement of the venous system (72%), 2 of the arterial system (14%), and 2 of both systems (14%). Of the 12 patients who had venous system involvement, 4 had thrombosis in the inferior vena cava (IVC), 2 in both the IVC and the hepatic vein, 1 in the IVC and splenic and portal veins, 1 in the IVC and hepatic and adrenal veins, 1 in the hepatic, portal, and renal veins, and 3 in the portal and superior mesenteric veins. Budd-Chiari syndrome developed in five of the nine patients who had thrombosis of the IVC or hepatic vein. Arterial thrombosis was noted in four patients, hepatic artery in two, aorta in one, renal artery in one, pancreatic arcade in one, and splenic artery in one, with infarct of multiple organs including the liver, jejunum, colon, kidney, and adrenal gland. Seven of the 14 patients (50%) manifested thrombosis or infarct of multiple extra-abdominal organs.
Conclusion: PAPS should be included in the differential diagnosis when CT demonstrates infarcts in multiple organs or patients have recurrent episodes of venous or arterial thrombosis.