Atherosclerosis is increasingly considered an immune system-mediated process of the vascular system. Autoantibodies, autoantigens, pro-inflammatory cytokines and infectious agents play a role in that process. Autoimmunity-related cardiovascular disease and atherosclerosis are important clinical problems. The reason for accelerated atherosclerosis in patients with autoimmune rheumatic diseases remains unclear. Some cases of autoimmunity-related cardiovascular disease may be more related to thrombosis than atherosclerosis. Although, mechanisms causing the increased risk are not precisely clarified, a combination of traditional and nontraditional risk factors, including inflammation appears to be of importance. Further studies are needed to determine whether these autoimmune rheumatic diseases are also associated with accelerated atherosclerosis and its manifestations.