Skip to main content
SpringerLink
Log in

Task Force Report on Catastrophic Antiphospholipid Syndrome

  • Chapter
  • First Online:
Antiphospholipid Syndrome

  • 639 Accesses

Abstract

The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and systemic inflammatory syndrome (SIRS). The hallmark of catastrophic APS (CAPS) is the histopathologic evidence of small vessel occlusions. At present, there are no studies on the pathophysiologic mechanisms of CAPS. A precipitating factor is reported in more than half of the patients. The first clinical manifestation at the time of the catastrophic episode is a pulmonary complication in 24% of the cases, a neurologic feature in 18% and a renal feature in 18%. Cerebral involvement is the most frequent cause of death followed by cardiac involvement, infection, and multiorgan failure. The first-line treatment should include the combination of anticoagulation against thrombosis plus glucocorticoids against the manifestations of SIRS plus plasma exchange and/or intravenous immunoglobulins to remove or block the antiphospholipid antibodies and the cytokines involved in the SIRS.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Asherson RA, Cervera R, de Groot PR, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12:530–4.

    Article  CAS  PubMed  Google Scholar 

  2. Bucciarelli S, Cervera R, Espinosa G, Gómez-Puerta JA, Ramos-Casals M, Font J. Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors. Autoimmun Rev. 2006;6:72–5.

    Article  PubMed  Google Scholar 

  3. Kitchens CS, Erkan D, Brandão LR, et al. Thrombotic storm revisited: preliminary diagnostic criteria suggested by the Thrombotic Storm Study Group. Am J Med. 2011;124:290–6.

    Article  PubMed  Google Scholar 

  4. Espinosa G, Cervera R, Asherson RA. Catastrophic antiphospholipid syndrome and sepsis: a common link? J Rheumatol. 2007;34:923–6.

    CAS  PubMed  Google Scholar 

  5. Cervera R, CAPS Registry Project Group. Catastrophic antiphospholipid syndrome (CAPS): update from the “CAPS Registry”. Lupus. 2010;19:412–8.

    Article  CAS  PubMed  Google Scholar 

  6. Cervera R, Bucciarelli S, Plasín MA, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun. 2009;32:240–5.

    Article  CAS  PubMed  Google Scholar 

  7. Bucciarelli S, Espinosa G, Cervera R, et al, for the CAPS Registry Project Group (European Forum on Antiphospholipid Antibodies). Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum 2006;54:2568–76.

    Google Scholar 

  8. Erkan D, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: updated diagnostic algorithms. Autoimmun Rev. 2010;10:74–9.

    Article  PubMed  Google Scholar 

  9. Bayraktar UD, Erkan D, Bucciarelli S, Espinosa G, Asherson R. The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J Rheumatol. 2007;34:346–52.

    PubMed  Google Scholar 

  10. Erkan D, Asherson RA, Espinosa G, et al. The long-term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis. 2003;62:530–3.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  11. Asherson RA, Espinosa G, Menahem S, et al. Relapsing catastrophic antiphospholipid syndrome: report of three cases. Semin Arthritis Rheum. 2008;37:366–72.

    Article  PubMed  Google Scholar 

  12. Asherson RA, Cervera R, Piette JC, et al. Catastrophic antiphospholipid syndrome: clinical and laboratory features of 50 patients. Medicine (Baltimore). 1998;77:195–207.

    Article  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;80:355–76.

    Article  CAS  Google Scholar 

  14. Asherson RA, Cervera R. Microvascular and microangiopathic antiphospholipid-associated syndromes (“MAPS”): semantic or antisemantic? Autoimmun Rev. 2008;7:164–7.

    Article  CAS  PubMed  Google Scholar 

  15. Espinosa G, Bucciarelli S, Cervera R, et al. Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies. Ann Rheum Dis. 2004;63:730–6.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  16. Espinosa G, Cervera R. Antiphospholipid syndrome: frequency, main causes and risk factors of mortality. Nat Rev Rheumatol. 2010;6:296–300.

    Article  CAS  PubMed  Google Scholar 

  17. Erkan D, Leibowitz E, Berman J, Lockshin MD. Perioperative medical management of antiphospholipid syndrome: hospital for special surgery experience, review of literature, and recommendations. J Rheumatol. 2002;29:843–9.

    PubMed  Google Scholar 

  18. Cervera R, Tektonidou MG, Espinosa G, et al. Task force on catastrophic antiphospholipid syndrome (APS) and non-criteria APS manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions. Lupus. 2011;20:165–73.

    Article  CAS  PubMed  Google Scholar 

  19. Szczepiorkowski ZM, Winters JL, Bandarenko N, et al. Guidelines on the use of therapeutic apheresis in clinical practice—evidence-based approach from the apheresis applications committee of the American Society for Apheresis. J Clin Apher. 2010;25:83–177.

    Article  PubMed  Google Scholar 

  20. Erkan D. Therapeutic and prognostic considerations in catastrophic antiphospholipid syndrome. Autoimmun Rev. 2006;6:98–103.

    Article  CAS  PubMed  Google Scholar 

  21. Raschi E, Testoni C, Bosisio D, et al. Role of the MyD88 transduction signaling pathway in endothelial activation by antiphospholipid antibodies. Blood. 2003;101:3495–500.

    Article  CAS  PubMed  Google Scholar 

  22. Zhang J, Lieske K, McCrae B, McCrae K. Activation of endotelial cells by β2glycoprotein I (β2GPI) antibodies is mediated by annexin II cross linking and may involve TLR4. Blood. 2004;104:83.

    Google Scholar 

  23. Andersen OM, Benhayon D, Curran T, Willnow TE. Differential binding of ligands to the apolipoprotein E receptor 2′. Biochemistry. 2003;42:9355–64.

    Article  CAS  PubMed  Google Scholar 

  24. Aguilar-Valenzuela R, Martínez-Martínez LA, Pierangeli SS. A comprehensive review of thrombogenic mechanisms in APS. Op Autoimmun J. 2010;2:58–66.

    Article  CAS  Google Scholar 

  25. Lämmle B, Kremer Hovinga JA, Alberio L. Thrombotic thrombocytopenic purpura. J Thromb Haemost. 2005;3:1663–75.

    Article  PubMed  Google Scholar 

  26. Matsuda J, Sanaka T, Gohchi K, et al. Occurrence of thrombotic thrombocytopenic purpura in a systemic lupus erythematosus patient with antiphospholipid antibodies in association with decreased activity of von Willebrand factor-cleaving protease. Lupus. 2002;11:463–4.

    Article  CAS  PubMed  Google Scholar 

  27. Mukai M, Ieko M, Atsumi T, Notoya A, Kohno M. Multiple thromboses in major arteries in a patient with antiphospholipid syndrome associated with excess of a large multimer of von Willebrand factor. Lupus. 2001;10:895–6.

    Article  CAS  PubMed  Google Scholar 

  28. Hillmen P, Young NS, Schubert J, et al. The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med. 2006;355:1233–43.

    Article  CAS  PubMed  Google Scholar 

  29. Locke JE, Magro CM, Singer AL, et al. The use of antibody to complement protein C5 for salvage treatment of severe antibody-mediated rejection. Am J Transplant. 2009;9:231–5.

    Article  CAS  PubMed  Google Scholar 

  30. Lonze BE, Singer AL, Montgomery RA. Eculizumab and renal transplantation in a patient with CAPS. N Engl J Med. 2010;362:1744–5.

    Article  CAS  PubMed  Google Scholar 

  31. Erkan D, Lockshin MD. New approaches for managing antiphospholipid syndrome. Nat Clin Pract Rheumatol. 2009;5:160–70.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

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

    Gerard Espinosa MD, PhD, Horacio Berman MD, PhD & Ricard Cervera MD, PhD

  2. The Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, Weill Cornell Medical College of Cornell University, New York, NY, USA

    Doruk Erkan MD

Authors
  1. Gerard Espinosa MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Horacio Berman MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Doruk Erkan MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ricard Cervera MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ricard Cervera MD, PhD .

Editor information

Editors and Affiliations

  1. The Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, New York, NY, USA

    Doruk Erkan

  2. University of Texas Medical Branch, 301 University Boulevard, Galveston, 77555-0883, Texas, USA

    Silvia S. Pierangeli

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Espinosa, G., Berman, H., Erkan, D., Cervera, R. (2012). Task Force Report on Catastrophic Antiphospholipid Syndrome. In: Erkan, D., Pierangeli, S. (eds) Antiphospholipid Syndrome. Springer, Boston, MA. https://doi.org/10.1007/978-1-4614-3194-7_12

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-3194-7_12

  • Published:

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4614-3193-0

  • Online ISBN: 978-1-4614-3194-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation