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| Name | Class |
|---|---|
| European Union | OTHER |
| University of Giessen | OTHER |
| University of Zurich | OTHER |
| University of Florence |
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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| no vasodilator | patients receiving no vasodilator | ||
| CCB | patients receiving a calcium channel blocker (CCB) for digital vasculopathy | ||
| i.v. prostanoids or PDE5i or ETRAs | patients treated with i.v. prostanoids or phosphodiesterase-5 inhibitors (PDE5i) or endothelin receptor antagonists (ETRA), regardless of whether a calcium channel blocker is given in addition |
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| Measure | Description | Time Frame |
|---|---|---|
| The number of patients with pulmonary hypertension at 2 years | 2 years |
| Measure | Description | Time Frame |
|---|---|---|
| Time to development of precapillary pulmonary hypertension | participants will be followed for the duration of 2 years, the time until development of precapillary pulmonary hypertension is the secondary outcome | 2 years |
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Inclusion Criteria:
ACR = American College of Rheumatology; EULAR = European League Against Rheumatism; PRES = Pediatric Rheumatology European Society
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The study population are adult and juvenile systemic sclerosis patients from the EUSTAR cohort (MEDSonline database) and the juvenile systemic sclerosis working group cohort
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Yannick Allanore, Prof. | Contact | yannick.allanore@cch.aphp.fr | ||
| Jérome Avouac, Prof. | Contact | javouac@yahoo.fr |
| Name | Affiliation | Role |
|---|---|---|
| Yannick Allanore, Prof. | Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016 | Principal Investigator |
| Ulf Müller-Ladner, Prof. | Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016 | Recruiting | Paris | 75014 | France |
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| ID | Term |
|---|---|
| D012595 | Scleroderma, Systemic |
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |
| D008171 | Lung Diseases |
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| OTHER |
| University of Campania Luigi Vanvitelli | OTHER |
| University of Basel | OTHER |
| University College, London | OTHER |
| Charite University, Berlin, Germany | OTHER |
| University of Pecs | OTHER |
| University of Leeds | OTHER |
| Schoen Klinik Hamburg Eilbek | OTHER |
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| Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology | Recruiting | Bad Nauheim | 61231 | Germany |
|
| Charité Universitätsmedizin Berlin, Charité Centrum 12 für Innere Medizin und Dermatologie, Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie | Recruiting | Berlin | 10117 | Germany |
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| Centre for Pediatric Rheumatology, Klinikum Eilbek | Recruiting | Hamburg | 22081 | Germany |
|
| Pecsi Tudomanyegyetem - University of Pecs | Recruiting | Pécs | H-7622 | Hungary |
|
| University of Florence, Denothe Centre, Division of Rheumatology AOUC, Department of Biomedicine | Recruiting | Florence | 50139 | Italy |
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| Policlinico, Via Pansini | Recruiting | Napoli-Italia | 5-80131 | Italy |
|
| Felix-Platter Spital, University of Basel | Recruiting | Basel | CH 4012 Basel | Switzerland |
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| University of Zurich, Department of Rheumatology | Recruiting | Zurich | 8006 | Switzerland |
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| The Universitiy of Leeds, Division of Rheumatic and Musculoskeletal Disease, St James's University Hospital | Recruiting | Leeds | LS9 7TF | United Kingdom |
|
| Royal Free Hospital, University College London | Recruiting | London | NW3 2QG | United Kingdom |
|
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |