Abstract
BackgroundCardiac involvement in systemic sclerosis (SSc) is associated with a variable phenotype including heart failure, arrhythmias and pulmonary hypertension. The aim of the present study was to evaluate clinical characteristics, histopathological findings and outcome of patients with SSc and a clinical phenotype suggesting cardiac involvement.Methods and Results25 patients with SSc and clinical signs of cardiac involvement were included between June 2007 and December 2010. They underwent routine clinical work-up including laboratory testing, echocardiography, left and right heart catheterization, holter recordings and endomyocardial biopsy. Primary endpoint (EP) was defined as the combination of cardiovascular death, arrhythmic endpoints (defined as appropriate discharge of implantable cardioverter defibrillator (ICD)) or rehospitalization due to heart failure. The majority of patients presented with slightly impaired left ventricular function (mean LVEF 54.1±9.0%, determined by echocardiography). Endomyocardial biopsies detected cardiac fibrosis in all patients with a variable area percentage of 8% to 32%. Cardiac inflammation was diagnosed as follows: No inflammation in 3.8%, isolated inflammatory cells in 38.5%, a few foci of inflammation in 30.8%, several foci of inflammation in 15.4%, and pronounced inflammation in 7.7% of patients. During follow up (FU) (22.5 months), seven (28%) patients reached the primary EP. Patients with subsequent events showed a higher degree of fibrosis and inflammation in the myocardium by trend. While patients with an inflammation grade 0 or 1 showed an event rate of 18.2%, the subgroup of patients with an inflammation grade 2 presented with an event rate of 25% versus an event rate of 50% in the subgroup of patients with an inflammation grade 3 and 4, respectively (p=0.193). Furthermore, the subgroup of patients with fibrosis grade 1 showed an event rate of 11%, patients with fibrosis grade 2 and 3 presented with an event rate of 33% and 42% respectively (p = 0.160).ConclusionsPatients with SSc and clinical signs of cardiac involvement presented with mildly impaired LVEF. Prognosis was poor with an event rate of 28% within 22.5 months FU and was associated with the degree of cardiac inflammation and fibrosis.
Highlights
Systemic sclerosis (SSc) is a rare chronic disease of unknown cause characterized by diffuse fibrosis, degenerative changes, and vascular abnormalities in the skin, joints, and internal organs including the heart
Prognosis was poor with an event rate of 28% within 22.5 months follow up (FU) and was associated with the degree of cardiac inflammation and fibrosis
Indications for endomyocardial biopsy were based on these clinical criteria mentioned above or one of the following clinical indications: new-onset heart failure of 2-week duration with dilation of the left ventricle and hemodynamic compromise, new-onset heart failure of up to 3-month duration with dilated left ventricle and malignant arrhythmias or failure to respond to usual care or suspected cardiac involvement with impaired global or regional systolic left or right ventricular function, enlargement of the left or right ventricle, pericardial effusion, myocardial hypertrophy, or abnormal myocardial echo patterns in transthoracic echocardiography (TTE) suggesting myocardial involvement of systemic sclerosis (SSc)
Summary
Systemic sclerosis (SSc) is a rare chronic disease of unknown cause characterized by diffuse fibrosis, degenerative changes, and vascular abnormalities in the skin, joints, and internal organs including the heart. Primary cardiac involvement in progressive SSc remains clinically silent for a long time period, but is associated with a very poor prognosis, when first symptoms become apparent [1,2,3]. Patients with limited (lc) or diffuse cutaneous (dc) SSc are characterized by clinically evident, inflammatory, and fibrotic processes of the skin. These mechanisms play a major role in cardiac involvement leading to various clinical manifestations like heart failure, arrhythmias and pulmonary hypertension. Cardiac involvement in systemic sclerosis (SSc) is associated with a variable phenotype including heart failure, arrhythmias and pulmonary hypertension. The aim of the present study was to evaluate clinical characteristics, histopathological findings and outcome of patients with SSc and a clinical phenotype suggesting cardiac involvement
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