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Prevention and Treatment of Thrombotic Antiphospholipid Syndrome

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Antiphospholipid Syndrome

Abstract

Antiphospholipid syndrome (APS) is a clinical syndrome characterized by macro- and/or microvascular thrombosis and pregnancy morbidity, with laboratory criteria including persistently positive lupus anticoagulant and/or anticardiolipin antibody and/or anti-β2 glycoprotein-1 antibody. Patients with antiphospholipid antibodies (aPL) appear to be at increased risk of thrombosis. Primary prophylaxis with aspirin is probably indicated to reduce the risk of a first event in selected patients with positive aPL, though data are limited; aspirin may also be the treatment of choice in selected low-risk patients with prior APS-associated stroke. Generally, secondary prevention of thrombosis involves the administration of anticoagulation as well as, similar to primary prophylaxis, identification and management of modifiable risk factors. Patients with aPL and a history of prior venous thromboembolism without arterial thrombosis are adequately treated with warfarin administered to a target INR of 2.0–3.0. There is a lack of good-quality evidence to support any particular therapeutic strategy for patients with a history of arterial thrombosis. Standard-intensity warfarin (INR 2.0–3.0) is used in many centers, though some centers may choose to use higher-intensity warfarin (INR ≥3.0). All patients with aPL (with/without APS) should engage in regular cardiovascular disease (CVD) screening programs according to national guidelines. Any such patient with an inflammatory rheumatic disease should be managed to minimize inflammatory disease activity, so-called treat-to-target approaches. Hydroxychloroquine is an additional therapy that may facilitate achieving treat-to-targets but may have additional CVD protective properties. Aspirin is frequently added, particularly to patients with “traditional” atherosclerotic risk factors and/or demonstrated atherosclerotic lesions. While a lower threshold for instituting additional CVD risk interventions seems reasonable, currently there is no consensus to support a particular threshold or risk adjustment.

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Author information

Authors and Affiliations

  1. Pathology and Molecular Medicine, St Joseph’s Hospital and McMaster University, Hamilton, ON, Canada

    Mark Crowther

  2. Department of Medicine, McMaster University, Division of Rheumatology, Hamilton, ON, Canada

    Kimberly J. Legault

  3. Department of Medicine, Division of Hematology, University of Washington Medical Center, Seattle, WA, USA

    David A. Garcia

  4. First Department of Propaedeutic Internal Medicine, Rheumatology Unit, Laiko Hospital, Athens, Greece

    Maria G. Tektonidou

  5. Autoimmune Diseases Research Unit, Internal Medicine Service, Cruces University Hospital, Barakaldo, Basque Country, Spain

    Amaia Ugarte & Guillermo Ruiz-Irastorza

  6. Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology Medicine and Health, The University of Manchester, Manchester, UK

    Ian N. Bruce

  7. Department of Rheumatology, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, USA

    Doruk Erkan

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  1. Mark Crowther
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  2. Kimberly J. Legault
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  3. David A. Garcia
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  4. Maria G. Tektonidou
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  5. Amaia Ugarte
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  6. Ian N. Bruce
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  7. Doruk Erkan
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  8. Guillermo Ruiz-Irastorza
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Correspondence to Mark Crowther .

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

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Crowther, M. et al. (2017). Prevention and Treatment of Thrombotic Antiphospholipid Syndrome. In: Erkan, D., Lockshin, M. (eds) Antiphospholipid Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-319-55442-6_11

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  • DOI: https://doi.org/10.1007/978-3-319-55442-6_11

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-55440-2

  • Online ISBN: 978-3-319-55442-6

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