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Antiphospholipid syndrome: frequency, main causes and risk factors of mortality

Nature Reviews Rheumatology volume 6, pages 296–300 (2010)Cite this article

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Abstract

The presence of antiphospholipid antibodies has been shown to be related to an increased risk of thrombotic events. In patients with definite antiphospholipid syndrome (APS), that is, those who have had thrombosis and at least two positive determinations of antiphospholipid antibodies, secondary thromboprophylaxis with long-term anticoagulation therapy results in a low rate of recurrent thrombotic events, ranging from 0.016 to 0.031 events per patient per year. Thrombotic complications are, however, the most common cause of death in APS. The mortality rate in a large European cohort of patients with APS during a 5-year study period was 5.3%, and up to 40% of the deaths in this cohort were attributed to severe thrombotic events such as myocardial infarction, stroke and pulmonary embolism. Catastrophic APS is an unusual form of the disease, being observed in less than 1% of reported cases of APS, which is associated with a much higher mortality rate than classical APS. The combined use of anticoagulation, corticosteroids, plasma exchange and intravenous immunoglobulin therapy could result in a dramatic reduction in mortality, by approximately 20%, in patients with catastrophic APS.

Key Points

  • According to a large European cohort study, the mortality rate of the antiphospholipid syndrome (APS) during a 5-year period was 5.3%

  • Thrombotic complications—mainly myocardial infarction, stroke and pulmonary embolism—are the most common cause of death in patients with APS

  • Catastrophic APS is present in less than 1% of reported cases of APS, but it is associated with a considerably higher mortality rate than classical APS

  • Combination therapy with anticoagulants, corticosteroids, plasma exchange and intravenous immunoglobulin could result in a 20% reduction in mortality associated with catastrophic APS

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References

  1. Miyakis, S. et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J. Thromb. Haemost. 4, 295–306 (2006).

    Article  CAS  Google Scholar 

  2. Cervera, R. et al. Antiphospholipid syndrome. Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 46, 1019–1027 (2002).

    Article  Google Scholar 

  3. Ruiz-Irastorza, G., Hunt, B. J. & Khamashta, M. A. A systematic review of secondary thromboprophylaxis in patients with antiphospholipid antibodies. Arthritis Rheum. 57, 1487–1495 (2007).

    Article  Google Scholar 

  4. Cervera, R. et al. Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicenter prospective study of 1,000 patients. Ann. Rheum. Dis. 68, 1428–1432 (2009).

    Article  CAS  Google Scholar 

  5. Bucciarelli, S., Espinosa, G. & Cervera, R. The CAPS registry: morbidity and mortality of the catastrophic antiphospholipid syndrome. Lupus 18, 905–912 (2009).

    Article  CAS  Google Scholar 

  6. Asherson, R. A. et al. Catastrophic antiphospholipid syndrome: clinical and laboratory features of 50 patients. Medicine (Baltimore) 77, 195–207 (1998).

    Article  CAS  Google Scholar 

  7. Asherson, R. A. et al. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore) 80, 355–376 (2001).

    Article  CAS  Google Scholar 

  8. Bucciarelli, S. et al. Mortality in catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum. 54, 2568–2576 (2006).

    Article  Google Scholar 

  9. Rosove, M. H. & Brewer, P. M. C. Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients. Ann. Intern. Med. 117, 303–308 (1992).

    Article  CAS  Google Scholar 

  10. Khamashta, M. A. et al. The management of thrombosis in the antiphospholipid antibody syndrome. N. Engl. J. Med. 332, 993–997 (1995).

    Article  CAS  Google Scholar 

  11. Ames, P. R. J. et al. Bleeding and re-thrombosis in primary antiphospholipid syndrome on oral anticoagulation. A 8-year longitudinal comparison with mitral valve replacement and inherited thrombophilia. Thromb. Haemost. 93, 694–699 (2005).

    Article  CAS  Google Scholar 

  12. Giron-Gonzalez, J. A. et al. Antiphospholipid syndrome and asymptomatic carriers of antiphospholipid antibody: prospective analysis of 404 individuals. J. Rheumatol. 31, 1560–1567 (2004).

    PubMed  Google Scholar 

  13. Schulman, S., Svenungsson, E. & Granqvist, S. Anticardiolipin antibodies predict early recurrence of thromboembolism and death among patients with venous thromboembolism following anticoagulant therapy. Am. J. Med. 104, 332–338 (1998).

    Article  CAS  Google Scholar 

  14. Ruiz-Irastorza, G. et al. Bleeding and recurrent thrombosis in definite antiphospholipid syndrome: analysis of a series of 66 patients treated with oral anticoagulation to a target INR of 3.5. Arch. Intern. Med. 162, 1164–1169 (2002).

    Article  Google Scholar 

  15. Erkan, D., Yazici, Y., Sobel, R. & Lockshin, M. D. Primary antiphospholipid syndrome: functional outcome after 10 years. J. Rheumatol. 27, 2817–2821 (2002).

    Google Scholar 

  16. Asherson, R. A. The catastrophic antiphospholipid syndrome. J. Rheumatol. 19, 508–512 (1992).

    CAS  PubMed  Google Scholar 

  17. Espinosa, G. et al. Morbidity and mortality in the catastrophic antiphospholipid syndrome: pathophysiology, causes of death, and prognostic factors. Semin. Thromb. Haemost. 34, 290–294 (2008).

    Article  Google Scholar 

  18. Espinosa, G., Cervera, R. & Asherson, R. A. Catastrophic antiphospholipid syndrome and sepsis. A common link? J. Rheumatol. 34, 923–926 (2007).

    CAS  PubMed  Google Scholar 

  19. Kitchens, C. S. Thrombotic storm: when thrombosis begets thrombosis. Am. J. Med. 104, 381–385 (1998).

    Article  CAS  Google Scholar 

  20. Ortega-Hernandez, O. D., Agmon-Levin, N., Blank, M., Asherson, R. A. & Shoenfeld, Y. The physiopathology of the catastrophic antiphospholipid (Asherson's) syndrome: compelling evidence. J. Autoimmun. 32, 1–6 (2009).

    Article  CAS  Google Scholar 

  21. Cervera, R. et al. Catastrophic antiphospholipid syndrome: lessons fron the “CAPS Registry”—A tribute to the late Josep Font. Ann. NY Acad. Sci. 1108, 448–456 (2007).

    Article  CAS  Google Scholar 

  22. Bayraktar, U. D. et al. The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J. Rheumatol. 34, 346–352 (2007).

    PubMed  Google Scholar 

  23. Asherson, R. A. et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 12, 530–534 (2003).

    Article  CAS  Google Scholar 

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  1. Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Villarroel, 170, Barcelona, 08036, Catalonia, Spain

    Gerard Espinosa & Ricard Cervera

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  1. Gerard Espinosa
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Correspondence to Ricard Cervera.

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Espinosa, G., Cervera, R. Antiphospholipid syndrome: frequency, main causes and risk factors of mortality. Nat Rev Rheumatol 6, 296–300 (2010). https://doi.org/10.1038/nrrheum.2010.47

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