Development and Prevention of Pulmonary Hypertension in Systemic Sclerosis
- Conditions
- Systemic SclerosisPulmonary Hypertension
- Registration Number
- NCT01840748
- Lead Sponsor
- University of Paris 5 - Rene Descartes
- Brief Summary
Systemic sclerosis (SSc) is an orphan, multiorgan disease affecting the connective tissue of the skin and several internal organs.
Pulmonary hypertension (PH) is a fatal disorder characterized by an increase in pulmonary vascular resistance, which leads to right ventricular failure. Despite being recently the object of greater attention and despite therapeutic advances, pulmonary hypertension due to SSc remains associated with a dismal 47 - 67% 3-year survival. Among SSc patients prospectively followed in the "European League Against Rheumatism Scleroderma Trials and Research" (EUSTAR) cohort, 26% of death was related to pulmonary hypertension. Although some previous data have suggested the protective effects of calcium channel blockers on the development of pulmonary hypertension, the potential preventive effects of vasodilators for the prevention of Pulmonary hypertension have not been determined yet. In addition to be considered routinely for the treatment of SSc-related pulmonary hypertension, prostanoids, endothelin receptor antagonists (ETRA) and Phosphodiesterase-5 inhibitors (PDE5i) can also be used for this indication.
This observational trial is one out of five observational trials of the collaborative project "To decipher the optimal management of systemic sclerosis" (DeSScipher).
Aim of this observational trial is:
- to compare the outcomes of adult and juvenile SSc patients who are at high risk of developing pulmonary hypertension and are receiving either different vasodilator treatments or no vasodilator treatment.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- UNKNOWN
- Sex
- All
- Target Recruitment
- 960
- Juvenile and adult Systemic sclerosis patients, with diagnosis according to the ACR/EULAR adult SSc criteria and PRES/ACR/EULAR juvenile SSc criteria respectively
- Patients at high risk of pulmonary hypertension with a Cochin Risk prediction score >/= 3
ACR = American College of Rheumatology; EULAR = European League Against Rheumatism; PRES = Pediatric Rheumatology European Society
Not provided
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method The number of patients with pulmonary hypertension at 2 years 2 years
- Secondary Outcome Measures
Name Time Method Time to development of precapillary pulmonary hypertension 2 years participants will be followed for the duration of 2 years, the time until development of precapillary pulmonary hypertension is the secondary outcome
Trial Locations
- Locations (11)
Charité Universitätsmedizin Berlin, Charité Centrum 12 für Innere Medizin und Dermatologie, Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie
🇩🇪Berlin, Germany
Policlinico, Via Pansini
🇮🇹Napoli-Italia, Italy
Centre for Pediatric Rheumatology, Klinikum Eilbek
🇩🇪Hamburg, 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
Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology
🇩🇪Bad Nauheim, Germany
Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016
🇫🇷Paris, France
Felix-Platter Spital, University of Basel
🇨🇭Basel, Switzerland
The Universitiy of Leeds, Division of Rheumatic and Musculoskeletal Disease, St James's University Hospital
🇬🇧Leeds, United Kingdom
University of Zurich, Department of Rheumatology
🇨🇭Zurich, Switzerland