Diagnosis and management of vascular disease in systemic sclerosis

Vascular disease is seen in virtually all patients with systemic sclerosis and presents several challenges to physicians caring for them. Early recognition of isolated pulmonary hypertension and scleroderma renal crisis may be keys to successful outcomes. Although complete reversal of vascular disease is usually not possible, the availability of calcium channel blocking agents for RP and isolated pulmonary hypertension and ACE inhibitors for hypertensive renal disease has improved the morbidity and mortality of these patients. Optimal management of the vascular events in systemic sclerosis will ultimately depend on a clearer understanding of their pathogenesis. Treatment may ultimately be directed at preventing the development of these vascular syndromes. This may occur through combined therapy directed at both the abnormal immune response and disregulated fibroblast function seen in the disease, as well at the abnormal vascular responses. Such therapies have yet to be identified.