Thrombotic manifestations, mainly venous thromboembolism (VTE) and stroke, are the most common and potentially life-threatening presentations of antiphospholipid syndrome (APS). The management of APS requires the assessment of the antiphospholipid antibodies (aPL) profile, of concurrent systemic lupus erythematosus or other systemic autoimmune diseases and the presence of risk factors for cardiovascular disease and bleeding. Anticoagulation with vitamin K antagonists (VKA) remains the cornerstone of therapy for thrombotic APS. As platelets play a central role in APS, low-dose aspirin is the first option for primary thromboprophylaxis in asymptomatic aPL carriers, and also plays a role as combination therapy with VKAs in arterial thrombosis. Treatment with direct oral anticoagulants (DOACs) could be considered in certain low-risk situations, although they are not recommended in patients with arterial thrombosis or triple positive aPL. Adjuvant therapies such as hydroxychloroquine and statins may be useful in complex settings such as thrombotic recurrences or high risk of bleeding. In this article, we review the evidence and the recommendations of the guidelines for the treatment of APS, and provide a critical and practical approach of its management from our clinical perspective. (c) 2024 Elsevier Espana, S.L.U. All rights reserved.
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Liverpool John Moores Univ, Univ Liverpool, Liverpool Ctr Cardiovasc Sci, Liverpool L7 8TX, England
Liverpool & Heart & Chest Hosp, Liverpool L7 8TX, England
Sapienza Univ Rome, Dept Clin Internal Anaesthesiol & Cardiovasc Sci, I-00185 Rome, ItalyLiverpool John Moores Univ, Univ Liverpool, Liverpool Ctr Cardiovasc Sci, Liverpool L7 8TX, England
Bucci, Tommaso
Menichelli, Danilo
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Sapienza Univ Rome, Dept Clin Internal Anaesthesiol & Cardiovasc Sci, I-00185 Rome, Italy
Sapienza Univ Rome, Dept Gen & Specialized Surg Paride Stefanini, I-00185 Rome, ItalyLiverpool John Moores Univ, Univ Liverpool, Liverpool Ctr Cardiovasc Sci, Liverpool L7 8TX, England
Menichelli, Danilo
Palumbo, Ilaria Maria
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Sapienza Univ Rome, Dept Clin Internal Anaesthesiol & Cardiovasc Sci, I-00185 Rome, Italy
Sapienza Univ Rome, Dept Gen & Specialized Surg Paride Stefanini, I-00185 Rome, ItalyLiverpool John Moores Univ, Univ Liverpool, Liverpool Ctr Cardiovasc Sci, Liverpool L7 8TX, England
Palumbo, Ilaria Maria
Pastori, Daniele
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Liverpool John Moores Univ, Univ Liverpool, Liverpool Ctr Cardiovasc Sci, Liverpool L7 8TX, England
Liverpool & Heart & Chest Hosp, Liverpool L7 8TX, England
Sapienza Univ Rome, Dept Clin Internal Anaesthesiol & Cardiovasc Sci, I-00185 Rome, Italy
Local Camerelle, IRCCS Neuromed, I-86077 Pozzilli, ItalyLiverpool John Moores Univ, Univ Liverpool, Liverpool Ctr Cardiovasc Sci, Liverpool L7 8TX, England
Pastori, Daniele
Ames, Paul R. J.
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Nova Univ Lisbon, Nova Med Sch, Immune Response & Vasc Dis, iNOVA,4Hlth, P-1099085 Lisbon, Portugal
Dumfries Royal Infirm, Dept Haematol, Cargenbridge DG2 8RX, EnglandLiverpool John Moores Univ, Univ Liverpool, Liverpool Ctr Cardiovasc Sci, Liverpool L7 8TX, England
Ames, Paul R. J.
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Lip, Gregory Y. H.
Pignatelli, Pasquale
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Sapienza Univ Rome, Dept Clin Internal Anaesthesiol & Cardiovasc Sci, I-00185 Rome, ItalyLiverpool John Moores Univ, Univ Liverpool, Liverpool Ctr Cardiovasc Sci, Liverpool L7 8TX, England
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Hosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, SpainHosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, Spain
Diaz-Cremades, Juan
Fernandez-Fuertes, Fernando
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Hosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, SpainHosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, Spain
Fernandez-Fuertes, Fernando
Ruano, Jose A.
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Hosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, SpainHosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, Spain
Ruano, Jose A.
Tapia, Manuel
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Hosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, SpainHosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, Spain
Tapia, Manuel
Soler, Santiago
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Hosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, SpainHosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, Spain
Soler, Santiago
Bosch, Jose M.
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Hosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, SpainHosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, Spain
Bosch, Jose M.
Caballero, Mar
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Hosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, SpainHosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, Spain
Caballero, Mar
Gonzalez-San Miguel, Jose D.
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Hosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, SpainHosp Univ Insular Gran Canaria, Serv Hematol & Hemoterapia, Las Palmas Gran Canaria, Spain
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Univ Michigan, Dept Internal Med, Div Rheumatol, Ann Arbor, MI 48109 USA
Univ Michigan, Dept Pediat, Div Pediat Rheumatol, Ann Arbor, MI 48109 USAUniv Michigan, Dept Internal Med, Div Rheumatol, Ann Arbor, MI 48109 USA
Madison, Jacqueline A.
Duarte-Garcia, Ali
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Mayo Clin, Dept Internal Med, Div Rheumatol, Rochester, MN USA
Mayo Clin, Robert D & Patricia E Kern Ctr Sci Healthcare Del, Rochester, MN USAUniv Michigan, Dept Internal Med, Div Rheumatol, Ann Arbor, MI 48109 USA
Duarte-Garcia, Ali
Zuo, Yu
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Univ Michigan, Dept Internal Med, Div Rheumatol, Ann Arbor, MI 48109 USAUniv Michigan, Dept Internal Med, Div Rheumatol, Ann Arbor, MI 48109 USA
Zuo, Yu
Knight, Jason S.
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Univ Michigan, Dept Internal Med, Div Rheumatol, Ann Arbor, MI 48109 USAUniv Michigan, Dept Internal Med, Div Rheumatol, Ann Arbor, MI 48109 USA