Unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndrome
Antiphospholipid syndrome (APS) is a highly prothrombotic autoimmune disease characterized by the persistent presence of antiphospholipid autoantibodies (aPL) in association with thrombotic or nonthrombotic macro- and microvascular manifestations and/or pregnancy complications. This review is restri...
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Elsevier
2025-02-01
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| Series: | Research and Practice in Thrombosis and Haemostasis |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2475037925000482 |
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| author | Deepa J. Arachchillage Mike Laffan |
| author_facet | Deepa J. Arachchillage Mike Laffan |
| author_sort | Deepa J. Arachchillage |
| collection | DOAJ |
| description | Antiphospholipid syndrome (APS) is a highly prothrombotic autoimmune disease characterized by the persistent presence of antiphospholipid autoantibodies (aPL) in association with thrombotic or nonthrombotic macro- and microvascular manifestations and/or pregnancy complications. This review is restricted to thrombotic APS. Since the publication of the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for APS, several authors have emphasized the difference between “classification” and “diagnosis” as a potential pitfall for clinicians. In addition to challenges associated with the diagnosis of APS, there are many unresolved areas in understanding pathogenesis and in the management of both thrombotic and obstetric APS. Although APS is an antibody-mediated autoimmune disease, secondary thrombosis prevention is achieved by anticoagulation, mainly with vitamin K antagonists, such as warfarin, rather than immunomodulation. Evidence is convincing for the use of vitamin K antagonists in triple-positive APS with venous thromboembolism. However, the best anticoagulant approach in the management of venous thromboembolism patients with single or dual positive aPL is not clear. Management of patients with stroke or arterial thrombosis with aPL remains a major unresolved issue, although some guidelines recommend the use of warfarin rather than antiplatelet therapy as the first-line treatment of stroke in APS. Recurrent thrombosis, despite therapeutic anticoagulation, remains a frequent problem and may be explained by the contribution of thrombo-inflammation in patients with thrombotic APS. In this narrative review, we discuss some of the unresolved issues in the diagnosis and management of thrombotic APS. |
| format | Article |
| id | doaj-art-990d8a443e4541bd87a60d0901f5e32b |
| institution | DOAJ |
| issn | 2475-0379 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Research and Practice in Thrombosis and Haemostasis |
| spelling | doaj-art-990d8a443e4541bd87a60d0901f5e32b2025-08-20T03:18:58ZengElsevierResearch and Practice in Thrombosis and Haemostasis2475-03792025-02-019210272410.1016/j.rpth.2025.102724Unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndromeDeepa J. Arachchillage0Mike Laffan1Centre for Haematology, Department of Immunology and Inflammation, Imperial College London, London, United Kingdom; Department of Haematology, Imperial College Healthcare NHS Trust, London, United Kingdom; Correspondence Deepa J. Arachchillage, Centre for Haematology Department of Immunology and Inflammation, 5th Floor, Commonwealth Building, Du Cane Road, London, W12 0NN, UK.Centre for Haematology, Department of Immunology and Inflammation, Imperial College London, London, United Kingdom; Department of Haematology, Imperial College Healthcare NHS Trust, London, United KingdomAntiphospholipid syndrome (APS) is a highly prothrombotic autoimmune disease characterized by the persistent presence of antiphospholipid autoantibodies (aPL) in association with thrombotic or nonthrombotic macro- and microvascular manifestations and/or pregnancy complications. This review is restricted to thrombotic APS. Since the publication of the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for APS, several authors have emphasized the difference between “classification” and “diagnosis” as a potential pitfall for clinicians. In addition to challenges associated with the diagnosis of APS, there are many unresolved areas in understanding pathogenesis and in the management of both thrombotic and obstetric APS. Although APS is an antibody-mediated autoimmune disease, secondary thrombosis prevention is achieved by anticoagulation, mainly with vitamin K antagonists, such as warfarin, rather than immunomodulation. Evidence is convincing for the use of vitamin K antagonists in triple-positive APS with venous thromboembolism. However, the best anticoagulant approach in the management of venous thromboembolism patients with single or dual positive aPL is not clear. Management of patients with stroke or arterial thrombosis with aPL remains a major unresolved issue, although some guidelines recommend the use of warfarin rather than antiplatelet therapy as the first-line treatment of stroke in APS. Recurrent thrombosis, despite therapeutic anticoagulation, remains a frequent problem and may be explained by the contribution of thrombo-inflammation in patients with thrombotic APS. In this narrative review, we discuss some of the unresolved issues in the diagnosis and management of thrombotic APS.http://www.sciencedirect.com/science/article/pii/S2475037925000482antiphospholipid syndromeimmunomodulationstrokethrombosisvitamin K antagonist |
| spellingShingle | Deepa J. Arachchillage Mike Laffan Unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndrome Research and Practice in Thrombosis and Haemostasis antiphospholipid syndrome immunomodulation stroke thrombosis vitamin K antagonist |
| title | Unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndrome |
| title_full | Unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndrome |
| title_fullStr | Unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndrome |
| title_full_unstemmed | Unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndrome |
| title_short | Unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndrome |
| title_sort | unresolved issues in the diagnosis and management of thrombotic antiphospholipid syndrome |
| topic | antiphospholipid syndrome immunomodulation stroke thrombosis vitamin K antagonist |
| url | http://www.sciencedirect.com/science/article/pii/S2475037925000482 |
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