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Table 1 Cardiovascular risk management in systemic lupus erythematosus and antiphospholipid syndrome [integrated and adapted from [2, 5, 6]]

From: Arterial thrombosis triggered by methotrexate-induced hyperhomocysteinemia in a systemic lupus erythematosus patient with antiphospholipid antibodies

1. SLE diagnosis

2. Check cardiovascular/thrombotic risk factors (at diagnosis and repeat according to individual patient characteristics and risk levels), including:

 - aPL antibodies (if present define the aPL-associated risk profile)

 - Traditional CVD risk factors (e.g., smoking, obesity, dyslipidemia, hypertension, diabetes, CVD family history)

 - SLE-specific CVD risk factors (disease duration, disease activity, renal disease, GCs, hyperhomocysteinemia)

 - Acquired hypercoagulability (e.g., major surgery/trauma, immobilization, pregnancy, malignancies, smoking, nephrotic syndrome)

 - Hereditary hypercoagulability (e.g., factor V Leiden, prothrombin mutation, protein C deficiency a, protein S deficiency a, antithrombin deficiency a, elevated factor VIII activity a, hyperhomocysteinemia a)

3. Correct any modifiable risk factor (lifestyle modifications, pharmacologic therapies) and:

 - Hydroxychloroquine is recommended for all SLE patients (unless contraindicated) and should also be considered to reduce the risk of CVD

 - Low disease activity (at the lowest possible GCs dose) should be maintained to also reduce CVD risk

 - In asymptomatic aPL carriers (no history of thrombosis or pregnancy complications) prophylactic treatment with LDA is recommended, particularly in high-risk aPL profile patients

 - In patients with definite APS, long-term VKA is recommended (if recurrent thrombosis despite adequate VKA treatment, an increase of INR target or addition of LDA should be considered)

  1. aPL Antiphospholipid, APS Antiphospholipid syndrome, CVD Cardiovascular disease, GCs Glucocorticoids, INR International normalised ratio, LDA Low-dose aspirin, SLE Systemic lupus erythematosus, VKA Vitamin K antagonists
  2. athese factors can be hereditary or acquired