INTRODUCTION: Anticoagulation is frequently required for patients with systemic lupus erythematosus (SLE), given their high prevalence of cardiovascular disease and thrombosis, due to the complexity of disease pathophysiology, some overlap with antiphospholipid syndrome (APS), comorbidities, prevalent cardiovascular risk factors and treatment complications.

AREAS COVERED: This article outlines the epidemiology and pathophysiology of cardiovascular disease and arterial and/or venous thrombosis in SLE, with/without APS. We discuss common cardiovascular comorbidities and thrombotic disorders that may present as a complication of SLE and/or APS and highlight recommendations in current guidelines for anticoagulation management, alongside relevant disease-specific considerations. We specifically comment on the use of direct oral anticoagulants (DOACs) for venous thromboembolism (VTE) in these patients.

EXPERT OPINION: Assessment of cardiovascular risk and aPL profile is paramount in SLE patients. While warfarin is preferred in high-risk APS patients, DOACs can be used in a selected group of SLE and/or APS patients with VTE and no prior history of arterial thrombosis. Initiating anticoagulation in the setting of Catastrophic APS (CAPS) can be extremely challenging. Knowledge gaps remain regarding the management of patients with recurrent arterial and/or venous thrombosis despite anticoagulation. Research is needed to optimize strategies to reduce thrombotic events in APS patients.