Cardiac involvement in systemic sclerosis: Multicentric study in Tunisia

Abstract

Systemic sclerosis (SSc) is a systemic autoimmune disease that is characterised by endothelial dysfunction resulting in a small-vessel vasculopathy, fibroblast dysfunction, and immunological abnormalities. Cardiac manifestations of SSc can affect all structures of the heart; the incidence of cardiac disease is estimated to be about 7% in localized lcSSc and 21% in diffuse cutaneous sclerosis (dcSSc) patients. We investigated the prevalence and incidence (at time of diagnosis and during follow-up) of various cardiovascular diseases in a large multicenter study cohort in Tunisian patients with SSc throughout 8 Departments of Internal Medicine. One hundred and eight cases of SSc were recorded according to American College of Rheumatology criteria, during a 15-years period. Prevalence of cardiovascular diseases at the time of the SSc diagnosis and incidences during follow-up was assessed using Echocardiography and Electrocardiography. They were 93 women and 15 men with an average age at SSc onset of approximately 46.9 years. Only 18 patients had limited cutaneous SSc. Cardiac manifestations were observed in 29 patients (27%) pericardial effusion in 9 patients (8.3%),myocardial infarction n = 4 (3.7%), pulmonary arterial hypertension n = 15 (13.8%),valvulopathy n = 7 (6.4%), cardiac conduction diseases n = 6 (5.5%), arrhythmias n = 3 (2.7%), cardiomyopathy n = 9 (8.3%). Patients with limited SSc had similar cardiac abnormalities to patients with diffuse SSc. The prevalence of cardiac involvement in SSc is variable and difficult to determine because of the diversity of cardiac manifestations and the type of diagnostic tools applied. In our study, we confirm the increased risk of several SSc cardiac complications. Screening for subclinical cardiac involvement with modern, sensitive tools provides an opportunity for earlier detection and treatment.