The heart in scleroderma

Just as scleroderma can affect multiple organ systems, the cardiac manifestations of the disease are diverse. Although only relatively recently recognized, the heart is a major organ involved in scleroderma and the presence of cardiac involvement generally portends poorly for the patient. Cardiac involvement can generally be divided into direct myocardial effects and the indirect effect of other organ involvement (ie, pulmonary hypertension and renal crisis). Direct myocardial disease includes myositis, cardiac failure, cardiac fibrosis, coronary artery disease, conduction system abnormalities, and pericardial disease. The involvement of the heart in scleroderma was first identified in 1926 by Heine [1], followed by Weiss and colleagues [2], who described nine cases of systemic sclerosis (SSc) with congestive heart failure; it was first postulated that cardiac fibrosis was the etiology. Historically, the cardiac manifestations of scleroderma have been confined to a progressive myocardial fibrosis that was observed on autopsy sectioning of the heart [1,2]. Since these initial observations, it has been established that SSc can involve the myocardium, coronary arteries, pericardium, and the conduction system.