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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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  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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