Pulmonary Hypertension, Systemic Sclerosis
Conditions
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.
Interventions
None listed
Sponsors
European Union
University of Giessen
University of Zurich
University of Florence
University of Campania Luigi Vanvitelli
University of Basel
University College, London
Charite University, Berlin, Germany
University of Pecs
University of Leeds
Schoen Klinik Hamburg Eilbek
University of Paris 5 - Rene Descartes
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
Inclusion criteria
* 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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The number of patients with pulmonary hypertension at 2 years | 2 years |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| 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 |
Countries
France, Germany, Hungary, Italy, Switzerland, United Kingdom
Contacts
Primary ContactYannick Allanore, Prof.
Backup ContactJérome Avouac, Prof.
Outcome results
None listed