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Antiphospholipid Syndrome

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Lupus Erythematosus

Abstract

The antiphospholipid syndrome (APS) is a disorder that is characterized by the presence of antibodies directed against phospholipid moieties in conjunction with venous and/or arterial thromboembolic events and/or obstetrical complications. There are other associated features including but not limited to seizures, thrombocytopenia and autoimmune hemolytic anemia, livedo reticularis, and renal disease. Rare complications include the catastrophic APS in which multiple clotting events occur simultaneously or in rapid succession. The antiphospholipid antibody syndrome can be seen by itself; primary APS, or in conjunction with other diseases such as systemic lupus erythematosus (SLE); secondary APS. This chapter will discuss the clinical manifestations and the management of the APS.

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Sources

  1. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295.

    Article  PubMed  CAS  Google Scholar 

  2. Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002;46:1019.

    Article  PubMed  Google Scholar 

  3. Asherson RA, Khamashta MA, Ordi-Ros J, et al. The “primary” antiphospholipid syndrome: major clinical and serological features. Medicine (Baltimore). 1989;68:366.

    CAS  Google Scholar 

  4. Mandreoli M, Zucchelli P. Renal vascular disease in patients with primary antiphospholipid antibodies. Nephrol Dial Transplant. 1993;8:1277.

    PubMed  CAS  Google Scholar 

  5. Goodnight SH. Antiphospholipid antibodies and thrombosis. Curr Opin Hematol. 1994;1:354.

    PubMed  CAS  Google Scholar 

  6. Asherson RA, Lioté F, Page B, et al. Avascular necrosis of bone and antiphospholipid antibodies in systemic lupus erythematosus. J Rheumatol. 1993;20:284.

    PubMed  CAS  Google Scholar 

  7. Tektonidou MG, Varsou N, Kotoulas G, et al. Cognitive deficits in patients with antiphospholipid syndrome: association with clinical, laboratory, and brain magnetic resonance imaging findings. Arch Intern Med. 2006;166:2278.

    Article  PubMed  Google Scholar 

  8. Sanna G, Bertolaccini ML, Khamashta MA. Unusual clinical manifestations of the antiphospholipid syndrome. Curr Rheumatol Rev. 2006;2:387.

    Article  Google Scholar 

  9. Muscal E, Brey RL. Neurologic manifestations of the antiphospholipid syndrome: integrating molecular and clinical lessons. Curr Rheumatol Rep. 2008;10:67.

    Article  PubMed  CAS  Google Scholar 

  10. Rosove MH, Brewer PM. Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients. Ann Intern Med. 1992;117:303.

    PubMed  CAS  Google Scholar 

  11. Tektonidou MG, Laskari K, Panagiotakos DB, Moutsopoulos HM. Risk factors for thrombosis and primary thrombosis prevention in patients with systemic lupus erythematosus with or without antiphospholipid antibodies. Arthritis Rheum. 2009;61:29.

    Article  PubMed  Google Scholar 

  12. Ruffatti A, Del Ross T, Ciprian M, et al. Risk factors for a first thrombotic event in antiphospholipid antibody carriers. A multicentre, retrospective follow-up study. Ann Rheum Dis. 2009;68:397.

    Article  PubMed  CAS  Google Scholar 

  13. Asherson RA, Cervera R, Piette JC, et al. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore). 2001;80:355.

    Article  CAS  Google Scholar 

  14. Santoro SA. Antiphospholipid antibodies and thrombotic predisposition: underlying pathogenetic mechanisms. Blood. 1994;83:2389.

    PubMed  CAS  Google Scholar 

  15. Wisløff F, Jacobsen EM, Liestøl S. Laboratory diagnosis of the antiphospholipid syndrome. Thromb Res. 2002;108:263.

    Article  PubMed  Google Scholar 

  16. Harris EN, Gharavi AE, Boey ML, et al. Anticardiolipin antibodies: detection by radioimmunoassay and association with thrombosis in systemic lupus erythematosus. Lancet. 1983;2:1211.

    Article  PubMed  CAS  Google Scholar 

  17. Ichikawa K, Khamashta MA, Koike T, et al. Beta 2-glycoprotein I reactivity of monoclonal anticardiolipin antibodies from patients with the antiphospholipid syndrome. Arthritis Rheum. 1994;37:1453.

    Article  PubMed  CAS  Google Scholar 

  18. Forastiero R, Martinuzzo M. Prothrombotic mechanisms based on the impairment of fibrinolysis in the antiphospholipid syndrome. Lupus. 2008;17:872.

    Article  PubMed  CAS  Google Scholar 

  19. Girardi G, Berman J, Redecha P, et al. Complement C5a receptors and neutrophils mediate fetal injury in the antiphospholipid syndrome. J Clin Invest. 2003;112:1644.

    PubMed  CAS  Google Scholar 

  20. Girardi G, Redecha P, Salmon JE. Heparin prevents antiphospholipid antibody-induced fetal loss by inhibiting complement activation. Nat Med. 2004;10:1222.

    Article  PubMed  CAS  Google Scholar 

  21. Shamonki JM, Salmon JE, Hyjek E, Baergen RN. Excessive complement activation is associated with placental injury in patients with antiphospholipid antibodies. Am J Obstet Gynecol. 2007;196:167.e1.

    Article  Google Scholar 

  22. Giles IP, Isenberg DA, Latchman DS, Rahman A. How do antiphospholipid antibodies bind beta2-glycoprotein I? Arthritis Rheum. 2003;48:2111.

    Article  PubMed  CAS  Google Scholar 

  23. Oku K, Atsumi T, Bohgaki M, et al. Complement activation in patients with primary antiphospholipid syndrome. Ann Rheum Dis. 2009;68:1030.

    Article  PubMed  CAS  Google Scholar 

  24. Goldberg SN, Conti-Kelly AM, Greco TP. A family study of anticardiolipin antibodies and associated clinical conditions. Am J Med. 1995;99:473.

    Article  PubMed  CAS  Google Scholar 

  25. Segal JB, Streiff MB, Hofmann LV, et al. Management of venous thromboembolism: a systematic review for a practice guideline. Ann Intern Med. 2007;146:211.

    PubMed  Google Scholar 

  26. Crowther MA, Ginsberg JS, Julian J, et al. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003;349:1133.

    Article  PubMed  CAS  Google Scholar 

  27. Finazzi G, Marchioli R, Brancaccio V, et al. A randomized clinical trial of high-intensity warfarin vs. conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS). J Thromb Haemost. 2005;3:848.

    Article  PubMed  CAS  Google Scholar 

  28. Moll S, Ortel TL. Monitoring warfarin therapy in patients with lupus anticoagulants. Ann Intern Med. 1997;127:177.

    PubMed  CAS  Google Scholar 

  29. Erkan D, Harrison MJ, Levy R, et al. Aspirin for primary thrombosis prevention in the antiphospholipid syndrome: a randomized, double-blind, placebo-controlled trial in asymptomatic antiphospholipid antibody-positive individuals. Arthritis Rheum. 2007;56:2382.

    Article  PubMed  CAS  Google Scholar 

  30. Alarcón-Segovia D, Boffa MC, Branch W, et al. Prophylaxis of the antiphospholipid syndrome: a consensus report. Lupus. 2003;12:499.

    Article  PubMed  Google Scholar 

  31. Erkan D, Merrill JT, Yazici Y, et al. High thrombosis rate after fetal loss in antiphospholipid syndrome: effective prophylaxis with aspirin. Arthritis Rheum. 2001;44:1466.

    Article  PubMed  CAS  Google Scholar 

  32. Ruiz-Irastorza G, Khamashta MA. The treatment of antiphospholipid syndrome: a harmonic contrast. Best Pract Res Clin Rheumatol. 2007;21:1079.

    Article  PubMed  CAS  Google Scholar 

  33. Ruiz-Irastorza G, Hunt BJ, Khamashta MA. A systematic review of secondary thromboprophylaxis in patients with antiphospholipid antibodies. Arthritis Rheum. 2007;57:1487.

    Article  PubMed  Google Scholar 

  34. Sacco RL, Adams R, Albers G, et al. Guidelines for prevention of stroke in patients with ischemic stroke or transient ischemic attack: a statement for healthcare professionals from the American Heart Association/American Stroke Association Council on Stroke: co-sponsored by the Council on Cardiovascular Radiology and Intervention: the American Academy of Neurology affirms the value of this guideline. Stroke. 2006;37:577.

    Article  PubMed  Google Scholar 

  35. Durand JM, Lefevre P, Kaplanski G, et al. Correction of thrombocytopenia with dapsone in the primary antiphospholipid syndrome. J Rheumatol. 1993;20:1777.

    PubMed  CAS  Google Scholar 

  36. Erkan D, Asherson RA, Espinosa G, et al. Long term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis. 2003;62:530.

    Article  PubMed  CAS  Google Scholar 

  37. Lockshin MD, Erkan D. Treatment of the antiphospholipid syndrome. N Engl J Med. 2003;349:1177.

    Article  PubMed  CAS  Google Scholar 

  38. Zar T, Kaplan AA. Predictable removal of anticardiolipin antibody by therapeutic plasma exchange (TPE) in catastrophic antiphospholipid antibody syndrome (CAPS). Clin Nephrol. 2008;70:77.

    PubMed  CAS  Google Scholar 

  39. Kasthuri RS, Roubey RA. Warfarin and the antiphospholipid syndrome: does one size fit all? Arthritis Rheum. 2007;57:1346.

    Article  PubMed  Google Scholar 

  40. Ruiz-Irastorza G, Khamashta MA, Hunt BJ, et al. Bleeding and recurrent thrombosis in definite antiphospholipid syndrome: analysis of a series of 66 patients treated with oral anticoagulation to a target international normalized ratio of 3.5. Arch Intern Med. 2002;162:1164.

    Article  PubMed  Google Scholar 

  41. Kumar D, Roubey RA. Use of rituximab in the antiphospholipid syndrome. Curr Rheumatol Rep. 2010;12(1):40–4.

    Article  PubMed  CAS  Google Scholar 

  42. Cervera R, Khamashta MA, Shoenfeld Y, et al. Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2009;68:1428.

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Bonnie L. Bermas M.D. .

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Bermas, B.L. (2012). Antiphospholipid Syndrome. In: Schur, P., Massarotti, E. (eds) Lupus Erythematosus. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-1189-5_15

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  • DOI: https://doi.org/10.1007/978-1-4614-1189-5_15

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