Development and Prevention of Severe Heart Disease in Systemic Sclerosis

• Conditions: Cardiac Arrhythmia; Congestive Heart Failure; Systemic Sclerosis; Cardiac Diseases; Heart Block

• Registration Number: NCT01829126
• Lead Sponsor: Gabriele Valentini

Brief Summary

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.

Detailed Description

Not available

Study Timeline

• Study Start: 2013-04
• Study First Submitted: 2013-04-08
• Study First Submitted QC: 2013-04-08
• Study First Posted: 2013-04-11
• Status Verified: 2013-11
• Last Update Submitted: 2013-11-07
• Last Update Posted: 2013-11-08
• Primary Completion: 2016-08
• Study Completion: 2016-08

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 765

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.

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Cumulative incidence of CB, VA, pacemaker implantation, congestive heart failure and sudden death	1 years	Cumulative incidence of cardiac blocks, ventricular arrhythmias, pacemaker implantation, congestive heart failure and sudden death.

Trial Locations

Locations (11)

Centre for Pediatric Rheumatology, Klinikum Eilbek; 🇩🇪 Hamburg, Germany

The Universitiy of Leeds, Division of Rheumatic and Musculoskeletal Disease, St James's University Hospital; 🇬🇧 Leeds, United Kingdom

Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016; 🇫🇷 Paris, France

Policlinico, Via Pansini; 🇮🇹 Napoli-Italia, Italy

Charité Universitätsmedizin Berlin, Charité Centrum 12 für Innere Medizin und Dermatologie, Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie; 🇩🇪 Berlin, Germany

Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology; 🇩🇪 Bad Nauheim, Germany

Pecsi Tudomanyegyetem - University of Pecs; 🇭🇺 Pecs, Hungary

University of Florence, Denothe Centre, Division of Rheumatology AOUC, Department of Biomedicine; 🇮🇹 Firenze, Italy

Royal Free Hospital, University College London; 🇬🇧 London, United Kingdom

University of Zurich, Department of Rheumatology; 🇨🇭 Zurich, Switzerland

Felix-Platter Spital, University of Basel; 🇨🇭 Basel, Switzerland