Preliminary Study of Cardiovascular Manifestations and Cardiac Severity Scale in 58 Patients with Systemic Sclerosis in Iran Using the Medsger Scale

Background: Cardiac involvement in systemic sclerosis (SSc) is more prevalent than previously thought. In this study, the frequency and severity of cardiovascular involvement were assessed in SSc patients referred to Firouzgar Hospital. Methods: Fifty-eight patients with SSc, selected from the data bank of SSc patients, were reviewed for the frequency and severity of 8 organ involvements in this case series. The preliminary severity scale, published by international SSc study groups, was employed for the determination of the severity grade in the cardiovascular system. In the cardiac scoring scale, grade 0 represents normal heart (no cardiac involvement), grade 1 denotes mild involvement [electrocardiography (ECG) conduction defect and a left ventricular ejection fraction (LVEF) of 45-49%)], grade 2 signifies moderate involvement (arrhythmia, LVEF = 40-44%), grade 3 indicates severe involvement (LVEF < 40%)], and grade 4 stands for end stage (congestive heart failure and arrhythmia requiring treatment). Results: In this study, 24 (41.4%) patients were in the diffuse cutaneous (dcSSc) subset. The female to male ratio was 10.5:1, and the mean duration from symptom onset to diagnosis was 7.35 years for the dcSSc subset and 8.41 years for the limited cutaneous (lcSSc) subset of disease, there being no significant difference. Cardiac involvement in this series was seen in 13 (22.4%) cases; and there was no significant difference in terms of frequency and severity between the two disease subgroups (p value = 0.96 and p value = 0.46 respectively). Conclusion: Our findings showed that the cardiac involvement in this series was infrequent and that there was no significant difference in the severity of cardiovascular involvement between the two subtypes of SSc in the late stage of the disease.