Development and Prevention of Severe Heart Disease in Systemic Sclerosis

Study Overview
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Brief Summary
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Systemic sclerosis is an orphan, multiorgan disease affecting the connective tissue of the skin and all internal organs. Cardiac involvement, mainly characterised by small intramyocardial coronary artery involvement and myocardial fibrosis, can cause the development of impaired diastolic ventricular filling, cardiac blocks and ventricular arrhythmias, and can ensue in congestive heart failure and sudden death. Until now, no drug has been proven to have a therapeutic effect on SSc myocardial disease on an evidence-based level. Short-term trials and retrospective studies have suggested a favourable and protective effect of calcium channel blockers and angiotensin converting enzyme inhibitors in patients with myocardial involvement. However, no data are presently available on the prevention and treatment of severe heart disease. This observational trial is part of the collaborative project DeSScipher, one out of five observational trials to decipher the optimal management of systemic sclerosis. Aim of this observational trial is to assess the efficacy and safety of calcium channel blockers and angiotensin converting enzyme inhibitors in asymptomatic SSc patients with cardiac involvement.

Conditions
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Systemic Sclerosis, Cardiac Diseases, Heart Block, Cardiac Arrhythmia, Congestive Heart Failure

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Juvenile and adult systemic sclerosis patients, with diagnosis according to the SSc ACR/EULAR criteria or the PRES/ACR/EULAR juvenile SSc criteria respectively Asymptomatic (for cardiac disease) systemic sclerosis patients at risk for severe heart disease with at least one of the following risk factors: male sex and/or DLCO lower than 80% and/or sPAP > 30 mmHg and/or synovitis and/or joint contractures and/or digital ulcers and/or proteinuria. Asymptomatic for cardiac disease is defined by patients without dyspnea NYHA >/= II, without palpitations and without bilateral leg edema. Exclusion Criteria: Any significant pulmonary parenchymal (FVC < 70% and/or DLCO < 70%), pulmonary vascular (estimated systolic PAP > 40 mmHg), gastrointestinal (malabsorption syndrome or paralytic ileus) or renal (serum creatinine level >1.2 mg/dl, dialysis or previous scleroderma renal crisis) involvement Patients with dyspnea class NYHA >/= II Patients with palpitations Patients with bilateral leg edema.
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| CCB<br>Patients receiving calcium channel blockers (CCB) | |
| ACEi<br>Patients receiving angiotensin converting enzyme inhibitors (ACEi) | |
| CCB and ACEi<br>Patients receiving calcium channel blockers (CCB) and angiotensin converting enzyme inhibitors (ACEi) | |
| No treatment<br>Patients not receiving calcium channel blockers (CCB) and/or angiotensin converting enzyme inhibitors (ACEi) | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Cumulative incidence of CB, VA, pacemaker implantation, congestive heart failure and sudden death | Cumulative incidence of cardiac blocks, ventricular arrhythmias, pacemaker implantation, congestive heart failure and sudden death. | 1 years |