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Catastrophic Antiphospholipid Syndrome

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Hughes Syndrome

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Summary

CAPS develops in a minority (1%) of patients with aPL and is characterized by acute, vascular occlusion involving three or more organs. The disorder is characterized by a diffuse thrombotic microvasculopathy with a predilection for kidney, lung, brain, heart, skin, and gastrointestinal tract. Treatment is empiric and, although mortality may approach 50%, outcomes appear best for patients that receive combinations of heparin anticoagulation, steroids, plasmapheresis, intravenous gammaglobulin, and, in the setting of SLE disease exacerbation, cyclophosphamide. The etiology of CAPS awaits clarification but likely involves the activation of vascular endothelium to express surface adhesion molecules and possibly tissue factor that interact with circulating cellular inflammatory cells, elements of the phospholipid-dependent coagulation factors, and platelets in the presence of aPL. Improved therapy awaits better understanding of the underlying immunologic, coagulation, and vascular pathology.

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Authors and Affiliations

  1. Department of Rheumatology, Hospital for Joint Diseases, New York University School of Medicine, New York, NY, USA

    H. Michael Belmont MD

Authors
  1. H. Michael Belmont MD
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Editors and Affiliations

  1. Lupus Research Unit, St Thomas’ Hospital, London, UK

    M. A. Khamashta MD, FRCP, PhD (Deputy Head) (Deputy Head)

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© 2006 Springer-Verlag London Limited

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Belmont, H.M. (2006). Catastrophic Antiphospholipid Syndrome. In: Khamashta, M.A. (eds) Hughes Syndrome. Springer, London. https://doi.org/10.1007/1-84628-009-5_16

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  • DOI: https://doi.org/10.1007/1-84628-009-5_16

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-85233-873-2

  • Online ISBN: 978-1-84628-009-2

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