Development and Prevention of Severe Heart Disease in Systemic Sclerosis

Gabriele Valentini

Status

Unknown

Conditions

Systemic Sclerosis

Cardiac Diseases

Cardiac Arrhythmia

Heart Block

Congestive Heart Failure

Study type

Observational

Funder types

Other

Identifiers

NCT01829126

HEALTH-F5-2012-305495-OT5

Details and patient eligibility

About

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.

Enrollment

765 estimated patients

Sex

All

Volunteers

No Healthy Volunteers

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.