The role of endothelial cells in autoimmune rheumatic disease

Vasculopathy is a generic feature of autoimmune rheumatic disease and there is substantial evidence that endothelial cell dysfunction has a role in pathogenesis and clinical manifestations of this challenging group of diseases. Endothelial cells (EC) are a target for injury and through their essential functional role in vascular homoeostasis, this has significant impact. In addition, the emerging recognition that EC are important regulators of other cell types and can differentiate into other relevant cell types has direct relevance. These aspects are reviewed with a focus on recent published evidence regarding the importance of EC in development, progression and treatment of autoimmune rheumatic disease. The potential role of the adaptive and innate immune system in causing endothelial cell damage, including anti-endothelial cell autoantibodies, will be reviewed. Recent advances in understanding how EC may differentiate into mesenchymal lineages and the interplay between physiological roles in healing or tissue repair and dysfunctional responses in acquired connective tissue disease will be reviewed.