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Advances in the diagnosis and management of scleroderma-related vascular complications.

Vascular disease is observed in virtually all patients with systemic sclerosis and presents several challenges for the clinician. The alterations in vascular tone noted with Raynaud's phenomenon may have corollaries in both the pathogenesis and management of systemic sclerosis-related internal organ complications. Early recognition of isolated pulmonary hypertension, coronary microcirculatory disease, and scleroderma renal involvement may be the keys to successful outcomes. Future trends in the management of systemic sclerosis may be directed at preventing the development of these vascular syndromes. This may occur through combined therapies directed at the abnormal immune response, disregulated fibroblast function, and resultant abnormal vascular response.