Autoimmune-associated thrombosis: mechanisms, population burden, and prevention strategies

Autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis, antiphospholipid syndrome, and systemic vasculitis, are associated with increased risk of thrombosis, accelerated atherosclerosis, cardiovascular-events, and premature mortality. Heparin-induced thrombocytopaenia and vaccine-induced thrombocytopaenia and thrombosis (VITT) or VITT like syndrome are autoimmune thrombotic disorders that do not have additional features of autoimmune diseases. We summarise the epidemiology, pathophysiology, diagnosis, and management of autoimmune thrombosis. Key mechanisms include autoantibody-mediated coagulation activation, endothelial dysfunction, complement activation, platelet activation, neutrophil extracellular-trap formation, and thrombo-inflammation, promoting thrombin generation, impaired fibrinolysis, and vascular injury. Traditional cardiovascular risk factors, such as smoking, obesity, hypertension, diabetes mellitus, and infection, further amplify thrombotic risk. Management requires integrated strategies combining anticoagulation, immunomodulatory therapy, cardiovascular-risk reduction, and long-term surveillance. Autoimmune thrombosis is an under-recognised contributor to cardiovascular disease and premature mortality across Europe. Earlier diagnosis, improved risk stratification, multidisciplinary care, and integration of autoimmune diseases into European cardiovascular prevention and health-system strategies are essential to reduce morbidity and premature mortality.

DJA is funded by Medical Research Council UK (MR/Z505274/1) and infrastructure support was provided by the NIHR Imperial Biomedical Research Centre.