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Anti-phospholipid Antibody Mechanisms of Thrombosis

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  • First Online:
Encyclopedia of Medical Immunology

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Synonyms

Autoimmune thrombosis

Definition

Anti-phospholipid antibodies (aPL) induce a prothrombotic diathesis, ultimately leading to clotting events through three main mechanisms: interference with coagulation proteins in the fluid phase, interaction with the cell types involved in the haemostatic balance, and complement activation. Placental thrombosis contributes to the pathogenesis of aPL-induced pregnancy complications together with defective placentation, decidual inflammation, and complement activation.

Introduction

Anti-phospholipid antibody syndrome (APS) is characterized by recurrent arterial and/or venous thrombosis and/or pregnancy morbidity associated with aPL detectable by anti-cardiolipin (aCL) and/or anti-β2 glycoprotein I (β2GPI) and/or lupus anticoagulant (LA) assays (Miyakis et al. 2006).

aPL are not only diagnostic autoantibodies but are believed to play a pathogenic role mediating several clinical manifestations of the syndrome. The persistent presence of aPL is a...

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References

  • Ag˘ar C, Gwendolyn MA, van Os GMA, Matthias Mörgelin M, Richard R, Sprenger RR, Marquart JA, Urbanus RT, Ronald HWM, Derksen RHWM, Joost CM, Meijers JCM, de Groot PG. β2-Glycoprotein I can exist in 2 conformations: implications for our understanding of the antiphospholipid syndrome. Blood. 2006;116:1336–43.

    Google Scholar 

  • Agostinis C, Biffi S, Garrovo C, Durigutto P, Lorenzon A, Bek A, Bulla R, Grossi C, Borghi MO, Meroni P, Tedesco F. In vivo distribution of β2 glycoprotein I under various pathophysiologic conditions. Blood. 2011;118:4231–8.

    PubMed  CAS  Google Scholar 

  • Allen KL, Fonseca FV, Betapudi V, Willard B, Zhang J, McCrae KR. A novel pathway for human endothelial cell activation by antiphospholipid/anti-β2 glycoprotein I antibodies. Blood. 2012;119:884–93.

    PubMed  CAS  PubMed Central  Google Scholar 

  • Bertolaccini ML. Antibodies to prothrombin. Lupus. 2012;21:729–31.

    PubMed  CAS  Google Scholar 

  • Boles J, Mackman N. Role of tissue factor in thrombosis in antiphospholipid antibody syndrome. Lupus. 2010;19:370–8.

    PubMed  CAS  PubMed Central  Google Scholar 

  • Fischetti F, Durigutto P, Pellis V, Debeus A, Macor P, Bulla R, Bossi F, Ziller F, Sblattero D, Meroni P, Tedesco F. Thrombus formation induced by antibodies to beta2-glycoprotein I is complement dependent and requires a priming factor. Blood. 2005;106:2340–6.

    PubMed  CAS  Google Scholar 

  • Giannakopoulos B, Passam F, Rahgozar S, Krilis SA. Current concepts on the pathogenesis of the antiphospholipid syndrome. Blood. 2007;109:422–30.

    PubMed  CAS  Google Scholar 

  • Mahler M, Norman GL, Meroni PL, Khamashta M. Autoantibodies to domain 1 of beta 2 glycoprotein 1: A promising candidate biomarker for risk management in antiphospholipid syndrome. Autoimmun Rev. 2012;12:313–7.

    PubMed  CAS  Google Scholar 

  • Martinez de la Torre Y, Locati M, Buracchi C, Dupor J, Cook DN, Bonecchi R, Nebuloni M, Rukavina D, Vago L, Vecchi A, Lira SA, Mantovani A. Protection against inflammation- and autoantibody-caused fetal loss by the chemokine decoy receptor D6. Proc Natl Acad Sci USA. 2007;104:2319–24.

    PubMed  CAS  PubMed Central  Google Scholar 

  • Martinez de la Torre Y, Pregnolato F, D'Amelio F, Grossi C, Di Simone N, Pasqualini F, Nebuloni M, Chen P, Pierangeli S, Bassani N, Ambrogi F, Borghi MO, Vecchi A, Locati M, Meroni PL. Anti-phospholipid induced murine fetal loss: novel protective effect of a peptide targeting the β2 glycoprotein I phospholipid-binding site. Implications for human fetal loss. J Autoimmun. 2012;38:209–15.

    Google Scholar 

  • Meroni PL, Tedesco F, Locati M, Vecchi A, Di Simone N, Acaia B, Pierangeli SS, Borghi MO. Anti-phospholipid antibody mediated fetal loss: still an open question from a pathogenic point of view. Lupus. 2010;19:453–6.

    PubMed  CAS  Google Scholar 

  • Meroni PL, Borghi M, Raschi E, Tedesco F. Pathogenesis of antiphospholipid syndrome: understanding the antibodies. Nat Rev Rheumatol. 2011;7:330–9.

    PubMed  CAS  Google Scholar 

  • Meroni PL, Raschi E, Grossi C, Pregnolato F, Trespidi L, Acaia B. Borghi MO Obstetric and vascular APS: same autoantibodies but different diseases? Lupus. 2012;21:708–10.

    PubMed  CAS  Google Scholar 

  • Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, Derksen RH, Groot DE, Koike T, Meroni PL, Reber G, Reber G, Shoenfeld Y, Tincani A, Vlachoyiannopoulos PG, Krilis SA. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295–306.

    PubMed  CAS  Google Scholar 

  • Pierangeli SS, Chen PP, Raschi E, Scurati S, Grossi C, Borghi MO, Palomo I, Harris EN, Meroni PL. Antiphospholipid antibodies and the antiphospholipid syndrome: pathogenic mechanisms. Semin Thromb Hemost. 2008;34:236–50.

    PubMed  CAS  Google Scholar 

  • Rand JH, Wu XX, Quinn AS, Taatjes DJ. The annexin A5-mediated pathogenic mechanism in the antiphospholipid syndrome: role in pregnancy losses and thrombosis. Lupus. 2010;19:60–9.

    Google Scholar 

  • Ruiz-Irastorza G, Crowther M, Branch W, Khamashta MA. Antiphospholipid syndrome. Lancet. 2010;376:1498–509.

    PubMed  CAS  Google Scholar 

  • Shoenfeld Y, Blank M, Cervera R, Font J, Raschi E, Meroni PL. Infectious origin of the antiphospholipid syndrome. Ann Rheum Dis. 2006;65:2–6.

    PubMed  CAS  PubMed Central  Google Scholar 

  • Shoenfeld Y, Meroni PL, Toubi E. Antiphospholipid syndrome and systemic lupus erythematosus: are they separate entities or just clinical presentations on the same scale? Curr Opin Rheumatol. 2009;21:495–500.

    PubMed  CAS  Google Scholar 

  • Tincani A, Spatola L, Prati E, Allegri F, Ferremi P, Cattaneo R, Meroni P, Balestrieri G. The anti-beta2-glycoprotein I activity in human anti-phospholipid syndrome sera is due to monoreactive low-affinity autoantibodies directed to epitopes located on native beta2-glycoprotein I and preserved during species’ evolution. J Immunol. 1996;157:5732–8.

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Clinical Sciences and Community Health, Division of Rheumatology, University of Milan, Istituto G. Pini and IRCCS Istituto Auxologico Italiano, Pzza. C. Ferrari, 1-20122, Milan, Italy

    Pier Luigi Meroni & Cecilia B. Chighizola

Authors
  1. Pier Luigi Meroni
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  2. Cecilia B. Chighizola
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Corresponding author

Correspondence to Pier Luigi Meroni .

Editor information

Editors and Affiliations

  1. Department of Biochemistry and Molecular Biology, Monash University, Clayton, Victoria, Australia

    Ian R. Mackay

  2. Center for Autoimmune Disease Research, Department of Molecular Microbiology and Immunology, Department of Pathology, Johns Hopkins University, Baltimore, MD, USA

    Noel R. Rose

  3. The Feinstein Institute for Medical Research, Manhasset, NY, USA

    Betty Diamond

  4. The Feinstein Institute for Medical Research, Manhasset, NY, USA

    Anne Davidson

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Meroni, P.L., Chighizola, C.B. (2014). Anti-phospholipid Antibody Mechanisms of Thrombosis. In: Mackay, I.R., Rose, N.R., Diamond, B., Davidson, A. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84828-0_420

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  • DOI: https://doi.org/10.1007/978-0-387-84828-0_420

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-84827-3

  • Online ISBN: 978-0-387-84828-0

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