Cardiovascular Manifestations In Systemic Sclerosis

Systemic sclerosis (SS) was first identified over two centuries ago. It is characterized by its skin manifestations. However, cardiac signs were described in 1943 that were results of the underlying vascular involvement or sclerotic involvement of the kidney or lung. Cardiac involvement is a poor prognostic factor, but diagnosis may be late or missing because of the frequent discrepancy between clinical manifestation and cardiac involvement, for this reason, resort to all available diagnostic procedures is recommended to achieve an early diagnosis. Cardiac signs include myocardial fibrosis, myocardial necrosis (ischemia), Conduction abnormalities such as left anterior hemiblock, systolic and diastolic dysfunction, pericarditis and pericardial effusion/tamponade (which is usually secondary to renal involvement). These abnormalities usually present with biventicular congestive heart failure, atrial and ventricular arrhythmias, ischemic heart disease and sudden cardiac death. Antimyosin scintigraphy is a valuable non-invasive method for early detection of clinically silent cardiac involvement in patients with systemic sclerosis, even in the absence of left ventricular dysfunction. In patients with positive antimyosin study, intense pharmacologic treatment with vasodilators may be warranted. Cardiac score is a semiquantitative measure of cardiac involvement that improves prediction of prognosis in systemic sclerosis. Using this method, cardiac involvement assessed yearly by a semi quantitative cardiac scoring technique (the sum of 2 variables, scored 0 or 1 for left axis deviation and 0 or 2 for moderate-large pericardial effusion) and outcome at 10 years were evaluated in 90 systemic sclerosis patients enrolled in a 3 years prospective drug trial. Higher cardiac score was more significantly related to survival than any other individual cardiopulmonary variable. Predicted 6 year survival was 79% in patients with a cardiac score of 0, 51% in those with a score of 1, 15% in those with a score of 2 and 0% in those with a score of