To update our previous literature review and management recommendations on risk stratification and primary thrombosis prophylaxis in persistently antiphospholipid antibody (aPL)-positive individuals.

The estimated annual thrombosis incident rate (ATIR) among aPL-positive individuals with or without systemic autoimmune disease (SAIDx) is 0 to 5.3%, probably very low (< 1%/year) in those with no other SAIDx and thrombosis risk factors. Risk stratification based on aPL profile, age, additional SAIDx, and traditional cardiovascular disease (CVD) or venous thrombosis risk factors is crucial to determine the risk of first thrombosis in aPL-positive patients. The protective effect of low-dose aspirin for primary thrombosis prophylaxis prevention is not supported by randomized controlled data. Hydroxychloroquine is protective against thrombosis in aPL-positive SLE patients; however, its role in aPL-positive individuals with other SAIDx remains uncertain. Statins downregulate proinflammatory and prothrombotic biomarkers among antiphospholipid syndrome (APS) patients and may have a role in aPL-positive individuals with high CVD risk. The optimal primary thrombosis prevention strategy in patients with clinically significant aPL profiles should include (a) regular screening and elimination of non-aPL thrombosis risk factors, (b) optimal management of concomitant SAIDx,