Management of Vasculopathy in Connective Tissue Disease

Vascular disease is integral to the pathogenesis of connective tissue diseases. Small vessel vasculitis underlies many clinical manifestations of systemic lupus erythematosus and complicates rheumatoid arthritis and other connective tissue diseases. In contrast, in systemic sclerosis, abnormal vasomotor activity and a proliferative arterial vasculopathy are prominent. Disorders of angiogenesis have been implicated in many connective tissue diseases, thromboembolic disease is common in antiphospholipid antibody syndrome and Behçet's disease, and the association between chronic systemic inflammatory diseases and accelerated atherosclerosis is now well-recognised. Emerging evidence suggests that agents prescribed as either immunomodulators or cardiovascular risk modifiers may be effective in both capacities. The clinical significance of vascular disease in connective tissue disease is emphasized by the observation that excess mortality is predominantly cardiovascular. We re-evaluate the paradigm of disease-modifying therapy in connective tissue disease with attention to the mechanisms of vascular disease and review therapeutic strategies by which these may be inhibited.