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Catastrophic Antiphospholipid Syndrome

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Autoimmune Diseases

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Abstract

Antiphospholipid syndrome (APS) is characterized by the development of arterial and/or venous thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies (aPL) anticardiolipin (aCL), anti-β2-glycoprotein I (anti-β2GPI), and lupus anticoagulant (LA). Catastrophic antiphospholipid syndrome (CAPS) is a very severe variant of the classic APS, with predominant and extensive small-vessels occlusion that causes multiple organs thrombosis. Multiple thrombotic events occur in a short period of time and frequently lead to a life-threatening condition because of multiorgan failure. The mortality rate is high, but an early diagnosis and treatment with anticoagulation, corticosteroids, plasma exchange, and intravenous immunoglobulin (IVIG) may reduce this rate.

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Authors and Affiliations

  1. Lupus Unit, The Rayne Institute, St. Thomas’ Hospital, Lambeth Palace Road, London, SE1 7EH, UK

    Maria J. Cuadrado

Authors
  1. Maria J. Cuadrado
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  2. Giovanni Sanna
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  3. Maria Laura Bertolaccini
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  4. Munther A. Khamashta
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Corresponding author

Correspondence to Maria J. Cuadrado .

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Editors and Affiliations

  1. Rayne Institute, Lupus Research Unit, St. Thomas' Hospital, London, SE1 7EH, United Kingdom

    Munther A. Khamashta

  2. Barcelona, Surgey, Hospital Clinic, Calle Villarroel, 170, Barcelona, 08036, Spain

    Manuel Ramos-Casals

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Cuadrado, M.J., Sanna, G., Bertolaccini, M.L., Khamashta, M.A. (2011). Catastrophic Antiphospholipid Syndrome. In: Khamashta, M., Ramos-Casals, M. (eds) Autoimmune Diseases. Springer, London. https://doi.org/10.1007/978-0-85729-358-9_3

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  • DOI: https://doi.org/10.1007/978-0-85729-358-9_3

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  • Publisher Name: Springer, London

  • Print ISBN: 978-0-85729-357-2

  • Online ISBN: 978-0-85729-358-9

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