ABSTRACT
Primary myocardial involvement is common in systemic sclerosis and affects the prognosis of the disease when it is clinically evident. The exact mechanism leading to the myocardial fibrosis in systemic sclerosis remains unknown. Its detection depends, at least in part, on the sensitivity of the diagnostic methods used. The aim of this review is to analyze the types and mechanism of abnormalities in the heart in scleroderma and also summarize the diagnostic methods recommended for the most prevalent ones. The available literature describes the most frequent cardiovascular abnormalities in systemic sclerosis patients to be: myocardial dysfunction, pericarditis, rhythm and, most frequent, conduction abnormalities, pulmonary arterial hypertension. Key points regarding screening, diagnosis, and treatment remain to be determined more accurately. Novel diagnostic techniques and multicenter studies should improve our understanding of organ involvement in scleroderma, which will hopefully ultimately result in improved outcomes.