Customize your JAMA Network experience by selecting one or more topics from the list below.
Answer: Antiphospholipid Antibody Syndrome
Antiphospholipid antibody syndrome (APS) is characterized by a state of hypercoagulability potentially resulting in thrombosis of all segments of the arterial or venous beds.1 It is associated with the presence of a specific group of autoantibodies called antiphospholipid antibodies, circulating immunoglobulins that cross-react with cell membrane phospholipids. The 2 main types of antiphospholipid antibodies are the anticardiolipin antibody and the lupus anticoagulant. These antibodies are found in 2% of the general population2 and in 30% to 40% of patients with systemic lupus erythematosus.3,4 The term APS has been used to describe a clinical complex of vascular occlusion and ischemic events in patients with circulating antiphospholipid antibodies. The Sapporo criteria require the positivity on 2 occasions, at least 6 weeks apart, of anticardiolipin antibody at medium-high titers or lupus anticoagulant5 but this was recently revised to 12 weeks by international consensus.6 the clinical manifestations most often documented in prior investigations are recurrent deep venous thrombosis, recurrent spontaneous fetal abortion, and cerebrovascular accidents.7,8 Hepatic involvement is the most common of the APS abdominal manifestations, followed by thrombotic events involving different branches of the intestinal vasculature.9 Other abdominal manifestations associated with APS include cholecystic or splenic infarction and acute pancreatitis. Computed tomography is considered the first line of investigation in patients with APS who present with abdominal symptoms. A wide range of computed tomographic findings have been described for abdominal manifestation with APS: vascular thrombosis, segmental parenchymal nonenhancement in the liver, spleen, or kidney, intestinal wall thickening, and mesenteric infiltration.10 The underlying cause is sometimes unclear at the time of surgery; the diagnosis of APS is then made during the postoperative hospital stay. The presentation may be nonspecific, and some of the conditions may be life threatening, necessitating a high index of suspicion for their early recognition. In addition, screening for antiphospholipid antibodies should be carried out in patients who present with hepatic vascular occlusion or unexplained signs of intestinal angina.
Return to Quiz Case.
Correspondence: Satoshi Ogiso, MD, Kyoto Medical Center, Department of Surgery, 1-1, Mukaihata-cho, Fukakusa, Fushimi-ku, Kyoto 612-855, Japan (email@example.com).
Accepted for Publication: May 19, 2010.
Author Contributions:Study concept and design: Ogiso, Hata, and Tsuchiya. Acquisition of data: Ogiso, Nishikawa, and Tsuchiya. Analysis and interpretation of data: Ogiso, Hata, and Tsuchiya. Drafting of the manuscript: Ogiso and Nishikawa. Critical revision of the manuscript for important intellectual content: Ogiso, Hata, Tsuchiya, and Tsuchiya. Administrative, technical, and material support: Ogiso and Tsuchiya. Study supervision: Hata and Tsuchiya.
Financial Disclosure: None reported.
Image of the Month—Diagnosis. Arch Surg. 2010;145(10):1018. doi:10.1001/archsurg.2010.221-b
Artificial Intelligence Resource Center