The heart and pulmonary vasculature in scleroderma: clinical features and pathobiology

For nearly 50 years there have been concerns that scleroderma affects the heart. The two main mechanisms that are thought to be involved are; a fibrotic process secondary to myocardial Raynaud phenomenon (RP) or an immune-mediated myocarditis. In the past year several studies have been published that confirm the frequent occurrence of subclinical cardiac abnormalities in scleroderma. These studies do not tend to support the myocardial RP theory and raise doubts about the prognostic significance of these minor abnormalities. Involvement of the lungs in the form of pulmonary hypertension affects between 7-50% of patients with scleroderma. Until the past year this has been regarded by most as an untreatable condition associated with an inexorable decline. Epoprostenol therapy has now been proven beneficial in a randomized clinical trial, and several other agents show promise in the treatment of this condition. Furthermore, it is becoming apparent that lung transplantation is as successful in patients with scleroderma, as it is in primary pulmonary hypertension.