EULAR recommendations for the management of antiphospholipid syndrome in adults

EULAR recommendations for the management of antiphospholipid syndrome in adults

15 May 2019 | Maria G Tektonidou, Laura Andreoli, Marteen Limper, Zahir Amoura, Ricard Cervera, Nathalie Costedoat-Chalumeau, Maria Jose Cuadrado, Thomas Dörner, Raquel Ferrer-Oliveras, Karen Hambly, Munther A Khamashta, Judith King, Francesca Marchiori, Pier Luigi Meroni, Marta Mosca, Vittorio Pengo, Luigi Raio, Guillermo Ruiz-Irastorza, Yehuda Shoenfeld, Ljudmila Stojanovich, Elisabet Svenungsson, Denis Wahl, Angela Tincani, Michael M Ward
The European League Against Rheumatism (EULAR) has developed evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. The recommendations are based on a systematic literature review and expert opinion, focusing on risk stratification, primary and secondary thromboprophylaxis, obstetric APS, and catastrophic APS. Key points include: 1. **Risk Stratification**: Identifying high-risk aPL profiles (lupus anticoagulant, double or triple aPL positivity, or persistently high aPL titres) is critical for thrombotic and obstetric event management. Other risk factors include coexistence of systemic autoimmune diseases, history of thrombotic events, and traditional cardiovascular risk factors. 2. **Primary Thromboprophylaxis**: Asymptomatic aPL carriers with high-risk profiles should receive low-dose aspirin (LDA). Patients with systemic lupus erythematosus (SLE) and no prior thrombotic events should also receive LDA. Non-pregnant women with a history of obstetric APS should consider LDA. 3. **Secondary Thromboprophylaxis**: Patients with definite APS and first venous thrombosis should receive vitamin K antagonists (VKA) with a target INR of 2-3. Rivaroxaban is not recommended for triple aPL positivity due to the high risk of recurrent events. For recurrent venous or arterial thrombosis, additional strategies such as increasing INR targets, adding LDA, or switching to low molecular weight heparin may be considered. 4. **Obstetric APS**: Women with high-risk aPL profiles but no thrombotic events should consider LDA during pregnancy. Those with a history of obstetric APS should receive combination treatment with LDA and prophylactic heparin. For recurrent pregnancy complications, increasing heparin dose, adding hydroxychloroquine, or low-dose prednisolone may be considered. 5. **Catastrophic APS**: Early diagnosis and management of infections and minimizing anticoagulation discontinuation are crucial. Combination therapy with glucocorticoids, heparin, and plasma exchange or intravenous immunoglobulin (IVIG) is recommended for first-line treatment. The recommendations aim to guide treatment in adults with APS, but high-quality evidence is limited, highlighting the need for more research.The European League Against Rheumatism (EULAR) has developed evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. The recommendations are based on a systematic literature review and expert opinion, focusing on risk stratification, primary and secondary thromboprophylaxis, obstetric APS, and catastrophic APS. Key points include: 1. **Risk Stratification**: Identifying high-risk aPL profiles (lupus anticoagulant, double or triple aPL positivity, or persistently high aPL titres) is critical for thrombotic and obstetric event management. Other risk factors include coexistence of systemic autoimmune diseases, history of thrombotic events, and traditional cardiovascular risk factors. 2. **Primary Thromboprophylaxis**: Asymptomatic aPL carriers with high-risk profiles should receive low-dose aspirin (LDA). Patients with systemic lupus erythematosus (SLE) and no prior thrombotic events should also receive LDA. Non-pregnant women with a history of obstetric APS should consider LDA. 3. **Secondary Thromboprophylaxis**: Patients with definite APS and first venous thrombosis should receive vitamin K antagonists (VKA) with a target INR of 2-3. Rivaroxaban is not recommended for triple aPL positivity due to the high risk of recurrent events. For recurrent venous or arterial thrombosis, additional strategies such as increasing INR targets, adding LDA, or switching to low molecular weight heparin may be considered. 4. **Obstetric APS**: Women with high-risk aPL profiles but no thrombotic events should consider LDA during pregnancy. Those with a history of obstetric APS should receive combination treatment with LDA and prophylactic heparin. For recurrent pregnancy complications, increasing heparin dose, adding hydroxychloroquine, or low-dose prednisolone may be considered. 5. **Catastrophic APS**: Early diagnosis and management of infections and minimizing anticoagulation discontinuation are crucial. Combination therapy with glucocorticoids, heparin, and plasma exchange or intravenous immunoglobulin (IVIG) is recommended for first-line treatment. The recommendations aim to guide treatment in adults with APS, but high-quality evidence is limited, highlighting the need for more research.
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