In this study, we demonstrated that total Annexin V+EVs and Annexin V+ EVs from different circulating cells or endothelial cells were significantly increased in both SLE-non-PAH and SLE-PAH patients compared to HCs, indicating the involvement of EVs, particularly the EVs with PS exposure, in the development of SLE. Here, ANXA5 is linked to systemic lupus erythematosus.