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

For nearly 50 years there have been concerns that scleroderma affects the h eart. The two main mechanisms that are thought to be involved are; a fibrot ic process secondary to myocardial Raynaud phenomenon (RP) or an immune-med iated myocarditis, In the past year several studies have been published tha t confirm the frequent occurrence of subclinical cardiac abnormalities in s cleroderma. These studies do not tend to support the myocardial RP theory a nd raise doubts about the prognostic significance of these minor abnormalit ies. Involvement of the lungs in the form of pulmonary hypertension affects betw een 7-50% of patients with scleroderma. Until the past year this has been r egarded by most as an untreatable condition associated with an inexorable d ecline. 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 transplantat ion is as successful in patients with scleroderma, as it is in primary pulm onary hypertension Curr Opin Rheumatol 2001, 13:495-499 (C) 2001 Lippincott Williams & Wilkins, Inc.