Skip to main content
SpringerLink
Log in

15th International Congress on Antiphospholipid Antibodies Task Force on Catastrophic Antiphospholipid Syndrome Report

  • Chapter
  • First Online:
Antiphospholipid Syndrome

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a systemic disorder, resulting in both thrombotic and non-thrombotic events. Thrombosis manifests mainly as thrombotic microangiopathy (TMA), although large-vessel thrombosis occurs in up to one third of patients. Non-thrombotic responses include activation of inflammatory processes, such as systemic inflammatory response syndrome or acute respiratory distress syndrome. Specific conditions may trigger these cascades in susceptible individuals who have acquired antiphospholipid antibodies (aPL). Several inflammatory cascades are hypothesized, including NF-kB activation by Toll-like receptor and the complement system, but so far no specific mechanism or defect has been identified as the main cause, and no common molecular pathway has been found specifically to cause the syndrome. During the 15th International Congress on aPL in 2016, the “Task Force on CAPS ” members presented updated evidence and relevant literature in their areas of expertise.

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 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
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. Cervera R, Rodríguez-Pintó I, Colafrancesco S, et al. 14th international congress on antiphospholipid antibodies task force report on catastrophic antiphospholipid syndrome. Autoimmun Rev. 2014;13:699–707.

    Article  PubMed  Google Scholar 

  2. Ortega-Hernández OD, Agmon-Levin N, Blank M, Asherson RA, Shoenfeld Y. The physiopathology of the catastrophic antiphospholipid (Asherson’s) syndrome: compelling evidence. J Autoimmun. 2009;32:1–6.

    Article  PubMed  Google Scholar 

  3. Asherson RA. The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment. Lupus. 1998;7(Suppl 2):S55–62.

    Article  PubMed  Google Scholar 

  4. Triplett DA, Asherson RA. Pathophysiology of the catastrophic antiphospholipid syndrome (CAPS). Am J Hematol. 2000;65:154–9.

    Article  CAS  PubMed  Google Scholar 

  5. Asherson RA, Shoenfeld Y. The role of infection in the pathogenesis of catastrophic antiphospholipid syndrome – molecular mimicry? J Rheumatol. 2000;27:12–4.

    CAS  PubMed  Google Scholar 

  6. Blank M, Krause I, Fridkin M, et al. Bacterial induction of autoantibodies to β2-glycoprotein-I accounts for the infectious etiology of antiphospholipid syndrome. J Clin Invest. 2002;109:797–804.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Gharavi AE, Pierangeli SS, Espinola RG, Liu X, Colden-Stanfield M, Harris EN. Antiphospholipid antibodies induced in mice by immunization with a cytomegalovirus-derived peptide cause thrombosis and activation of endothelial cells in vivo. Arthritis Rheum. 2002;46:545–52.

    Article  CAS  PubMed  Google Scholar 

  8. Loizou S, Cazabon JK, Walport MJ, Tait D, So AKL. Similarities of specificity and cofactor dependence in serum antiphospholipid antibodies from patients with human parvovirus B19 infection and from those with systemic lupus erythematosus. Arthritis Rheum. 1997;40:103–98.

    Article  CAS  PubMed  Google Scholar 

  9. Garcia-Carrasco M, Mendoza-Pinto C, Macias-Diaz S, et al. The role of infectious diseases in the catastrophic antiphospholipid syndrome. Autoimmun Rev. 2015;14:1066–71.

    Article  CAS  PubMed  Google Scholar 

  10. 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 

  11. Kronbichler A, Frank R, Kirschfink M, et al. Efficacy of eculizumab in a patient with immunoadsorption-dependent catastrophic antiphospholipid syndrome: a case report. Medicine (Baltimore). 2014;93:e143.

    Article  CAS  Google Scholar 

  12. Strakhan M, Hurtado-Sbordoni M, Galeas N, Bakirhan K, Alexis K, Elrafei T. 36-year-old female with catastrophic antiphospholipid syndrome treated with eculizumab: a case report and review of literature. Case Rep Hematol. 2014;2014:704371.

    PubMed  PubMed Central  Google Scholar 

  13. Zikos TA, Sokolove J, Ahuja N, Berube C. Eculizumab induces sustained remission in a patient with refractory primary catastrophic antiphospholipid syndrome. J Clin Rheumatol. 2015;21:311–3.

    Article  PubMed  Google Scholar 

  14. 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  PubMed Central  Google Scholar 

  15. Agmon-Levin N, Rosário C, Katz B-SP, et al. Ferritin in the antiphospholipid syndrome and its catastrophic variant (cAPS). Lupus. 2013;22:1327–35.

    Article  CAS  PubMed  Google Scholar 

  16. Giannakopoulos B, Krilis SA. The pathogenesis of the antiphospholipid syndrome. N Engl J Med. 2013;368:1033–44.

    Article  CAS  PubMed  Google Scholar 

  17. Rodríguez-Pintó I, Moitinho M, Santacreu I, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev. 2016;15:1120–4.

    Article  PubMed  Google Scholar 

  18. Kitchens CS, Erkan D, Brandao 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 

  19. Manco-Johnson MJ, Wang M, Goldenberg NA, et al. Treatment, survival, and thromboembolic outcomes of thrombotic storm in children. J Pediatr. 2012;161:682–8. e1

    Article  PubMed  Google Scholar 

  20. Kitchens CS. Thrombotic storm: when thrombosis begets thrombosis. Am J Med. 1998;104:381–5.

    Article  CAS  PubMed  Google Scholar 

  21. Ortel TL, Erkan D, Kitchens CS. How I treat catastrophic thrombotic syndromes. Blood. 2015;126:1285–93.

    Article  CAS  PubMed  Google Scholar 

  22. Sarode R. Atypical presentations of thrombotic thrombocytopenic purpura: a review. J Clin Apher. 2009;24:47–52.

    Article  PubMed  Google Scholar 

  23. Kalish Y, Rottenstreich A, Rund D, Hochberg-Klein S. Atypical presentations of thrombotic thrombocytopenic purpura: a diagnostic role for ADAMTS13. J Thromb Thrombolysis. 2016;42:155–60.

    Google Scholar 

  24. Warkentin TE, Basciano PA, Knopman J, Bernstein RA. Spontaneous heparin-induced thrombocytopenia syndrome: two new cases and a proposal for defining this disorder. Blood. 2014;123:3651–4.

    Article  CAS  PubMed  Google Scholar 

  25. Miesbach W, Asherson RA, Cervera R, et al. The catastrophic antiphospholipid (Asherson’s) syndrome and malignancies. Autoimmun Rev. 2006;6:94–7.

    Article  CAS  PubMed  Google Scholar 

  26. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371:654–66.

    Article  CAS  PubMed  Google Scholar 

  27. Rodríguez-Pintó I, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: the current management approach. Best Pract Res Clin Rheumatol. 2016;30:239–49.

    Article  PubMed  Google Scholar 

  28. Raso S, Sciascia S, Kuzenko A, et al. Bridging therapy in antiphospholipid syndrome and antiphospholipid antibodies carriers: case series and review of the literature. Autoimmun Rev. 2015;14:36–42.

    Article  CAS  PubMed  Google Scholar 

  29. Vora SK, Asherson RA, Erkan D. Catastrophic antiphospholipid syndrome J Intensive Care Med. 2006;21:144–59.

    Google Scholar 

  30. Bucciarelli S, Espinosa G, Cervera R, et al. Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum. 2006;54:2268–76.

    Article  Google Scholar 

  31. Espinosa G, Cervera R. Current treatment of antiphospholipid syndrome: lights and shadows. Nat Rev Rheumatol. 2015;11:586–96.

    Article  CAS  PubMed  Google Scholar 

  32. Pons-Estel GJ, Salerni GE, Serrano RM, et al. Therapeutic plasma exchange for the management of refractory systemic autoimmune diseases: report of 31 cases and review of the literature. Autoimmun Rev. 2011;10:679–84.

    Article  PubMed  Google Scholar 

  33. Bayraktar UD, Erkan D, Bucciarelli S, et al. The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J Rheumatol. 2007;34:346–52.

    PubMed  Google Scholar 

  34. Berman H, Rodríguez-Pintó I, Cervera R, et al. Rituximab use in the catastrophic antiphospholipid syndrome: descriptive analysis of the CAPS registry patients receiving rituximab. Autoimmun Rev. 2013;12:1085–90.

    Article  CAS  PubMed  Google Scholar 

  35. Kronbichler A, Frank R, Kirschfink M, et al. Efficacy of eculizumab in a patient with immunoadsorption-dependent catastrophic antiphospholipid syndrome. Medicine (Baltimore). 2014;93:e143.

    Article  CAS  Google Scholar 

  36. Cervera R, Rodriguez-Pinto I. Catastrophic antiphospholipid syndrome: task force report summary. Lupus. 2014;23:1283–5.

    Article  PubMed  Google Scholar 

  37. Bontadi A, Ruffatti A, Falcinelli E, et al. Platelet and endothelial activation in catastrophic and quiescent antiphospholipid syndrome. Thromb Haemost. 2013;109:901–8.

    Article  CAS  PubMed  Google Scholar 

  38. Rodríguez-Pintó I, Santacreu I, Cervera R, Espinosa G. What is the best strategy in treating catastrophic antiphospholipid syndrome? Int J Clin Rheum Dis. 2015;10:245–55.

    Article  Google Scholar 

  39. Legault K, Hills C, Akl E, et al. Guideline for diagnosis and management of catastrophic APS. Lupus. 2016;25:8. (abstract)

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain

    Ricard Cervera, Ignasi Rodríguez Pintó & Gerard Espinosa

  2. Rabin Medical Center, Campus Beilinson, Medicine F- Recanati, Petah-Tikva, Israel

    Tamir Shragai

  3. Sheba Medical Center, Zabludowizc Center for Autoimmune Diseases, Tel-Hashomer, Ramat Gan, Israel

    Miri Blank

  4. Department of Autoimmunity, Sheba Medical Center, Tel Hashomer, Israel

    Yehuda Shoenfeld

  5. Rabin Medical Center, Internal Medicine F-Recanati, Petah-Tiqva, Israel

    Ilan Krause

  6. Department of Medicine, Duke University Medical Center, Durham, NC, USA

    Thomas L. Ortel

Authors
  1. Ricard Cervera
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ignasi Rodríguez Pintó
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Gerard Espinosa
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Tamir Shragai
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Miri Blank
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Yehuda Shoenfeld
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Ilan Krause
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Thomas L. Ortel
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ricard Cervera .

Editor information

Editors and Affiliations

  1. The Barbara Volcker Center for Women and Rheumatic Diseases, Associate Attending Physician, Hospital for Special Surgery, Associate Professor of Medicine, Weill Cornell Medicine, New York, New York, USA

    Doruk Erkan

  2. The Barbara Volcker Center for Women and Rheumatic Diseases, Attending Physician, Hospital for Special Surgery, Professor of Medicine and Obstetrics-Gynecology, Weill Cornell Medicine, New York, New York, USA

    Michael D. Lockshin

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing AG

About this chapter

Cite this chapter

Cervera, R. et al. (2017). 15th International Congress on Antiphospholipid Antibodies Task Force on Catastrophic Antiphospholipid Syndrome Report. In: Erkan, D., Lockshin, M. (eds) Antiphospholipid Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-319-55442-6_17

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-55442-6_17

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-55440-2

  • Online ISBN: 978-3-319-55442-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation