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Arch Surg. 2010;145(10):1018. doi:10.1001/archsurg.2010.221-b.
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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.

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Correspondence: Satoshi Ogiso, MD, Kyoto Medical Center, Department of Surgery, 1-1, Mukaihata-cho, Fukakusa, Fushimi-ku, Kyoto 612-855, Japan (oggy_so@ybb.ne.jp).

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.

Khamashta  MABertolaccini  MLHughes  GR Antiphospholipid (Hughes) syndrome. Autoimmunity 2004;37 (4) 309- 312
PubMed
Ford  SEKennedy  LFord  PM Clinicopathologic correlations of antiphospholipid antibodies: an autopsy study. Arch Pathol Lab Med 1994;118 (5) 491- 495
PubMed
Love  PESantoro  SA Antiphospholipid antibodies: anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders: prevalence and clinical significance. Ann Intern Med 1990;112 (9) 682- 698
PubMed
Harris  ENGharavi  AEHughes  GR Anti-phospholipid antibodies. Clin Rheum Dis 1985;11 (3) 591- 609
PubMed
Lockshin  MDSammaritano  LRSchwartzman  S Validation of the Sapporo criteria for antiphospholipid syndrome. Arthritis Rheum 2000;43 (2) 440- 443
PubMed
Miyakis  SLockshin  MDAtsumi  T  et al.  International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006;4 (2) 295- 306
PubMed
Insko  EKHaskal  ZJ Antiphospholipid syndrome: patterns of life-threatening and severe recurrent vascular complications. Radiology 1997;202 (2) 319- 326
PubMed
Provenzale  JMOrtel  TL Anatomic distribution of venous thrombosis in patients with antiphospholipid antibody: imaging findings. AJR Am J Roentgenol 1995;165 (2) 365- 368
PubMed
Uthman  IKhamashta  M The abdominal manifestations of the antiphospholipid syndrome. Rheumatology (Oxford) 2007;46 (11) 1641- 1647
PubMed
Kaushik  SFederle  MPSchur  PHKrishnan  MSilverman  SGRos  PR Abdominal thrombotic and ischemic manifestations of the antiphospholipid antibody syndrome: CT findings in 42 patients. Radiology 2001;218 (3) 768- 771
PubMed

Figures

Tables

References

Khamashta  MABertolaccini  MLHughes  GR Antiphospholipid (Hughes) syndrome. Autoimmunity 2004;37 (4) 309- 312
PubMed
Ford  SEKennedy  LFord  PM Clinicopathologic correlations of antiphospholipid antibodies: an autopsy study. Arch Pathol Lab Med 1994;118 (5) 491- 495
PubMed
Love  PESantoro  SA Antiphospholipid antibodies: anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders: prevalence and clinical significance. Ann Intern Med 1990;112 (9) 682- 698
PubMed
Harris  ENGharavi  AEHughes  GR Anti-phospholipid antibodies. Clin Rheum Dis 1985;11 (3) 591- 609
PubMed
Lockshin  MDSammaritano  LRSchwartzman  S Validation of the Sapporo criteria for antiphospholipid syndrome. Arthritis Rheum 2000;43 (2) 440- 443
PubMed
Miyakis  SLockshin  MDAtsumi  T  et al.  International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006;4 (2) 295- 306
PubMed
Insko  EKHaskal  ZJ Antiphospholipid syndrome: patterns of life-threatening and severe recurrent vascular complications. Radiology 1997;202 (2) 319- 326
PubMed
Provenzale  JMOrtel  TL Anatomic distribution of venous thrombosis in patients with antiphospholipid antibody: imaging findings. AJR Am J Roentgenol 1995;165 (2) 365- 368
PubMed
Uthman  IKhamashta  M The abdominal manifestations of the antiphospholipid syndrome. Rheumatology (Oxford) 2007;46 (11) 1641- 1647
PubMed
Kaushik  SFederle  MPSchur  PHKrishnan  MSilverman  SGRos  PR Abdominal thrombotic and ischemic manifestations of the antiphospholipid antibody syndrome: CT findings in 42 patients. Radiology 2001;218 (3) 768- 771
PubMed

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