Development and Prevention of Pulmonary Hypertension in Systemic Sclerosis

NCT ID: NCT01840748

Last Updated: 2013-04-26

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

960 participants

Study Classification

OBSERVATIONAL

Study Start Date

2013-04-30

Study Completion Date

2017-06-30

Brief Summary

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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.

Detailed Description

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Conditions

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Systemic Sclerosis Pulmonary Hypertension

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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no vasodilator

patients receiving no vasodilator

No interventions assigned to this group

CCB

patients receiving a calcium channel blocker (CCB) for digital vasculopathy

No interventions assigned to this group

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

No interventions assigned to this group

Eligibility Criteria

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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
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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European Union

OTHER

Sponsor Role collaborator

University of Giessen

OTHER

Sponsor Role collaborator

University of Zurich

OTHER

Sponsor Role collaborator

University of Florence

OTHER

Sponsor Role collaborator

University of Campania Luigi Vanvitelli

OTHER

Sponsor Role collaborator

University of Basel

OTHER

Sponsor Role collaborator

University College, London

OTHER

Sponsor Role collaborator

Charite University, Berlin, Germany

OTHER

Sponsor Role collaborator

University of Pecs

OTHER

Sponsor Role collaborator

University of Leeds

OTHER

Sponsor Role collaborator

Schoen Klinik Hamburg Eilbek

OTHER

Sponsor Role collaborator

University of Paris 5 - Rene Descartes

OTHER

Sponsor Role lead

Responsible Party

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Yannick ALLANORE

Prof. Yannik Allanore

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Yannick Allanore, Prof.

Role: PRINCIPAL_INVESTIGATOR

Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016

Ulf Müller-Ladner, Prof.

Role: STUDY_CHAIR

Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology

Locations

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Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016

Paris, , France

Site Status RECRUITING

Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology

Bad Nauheim, , Germany

Site Status RECRUITING

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

Berlin, , Germany

Site Status RECRUITING

Centre for Pediatric Rheumatology, Klinikum Eilbek

Hamburg, , Germany

Site Status RECRUITING

Pecsi Tudomanyegyetem - University of Pecs

Pécs, , Hungary

Site Status RECRUITING

University of Florence, Denothe Centre, Division of Rheumatology AOUC, Department of Biomedicine

Florence, , Italy

Site Status RECRUITING

Policlinico, Via Pansini

Napoli-Italia, , Italy

Site Status RECRUITING

Felix-Platter Spital, University of Basel

Basel, , Switzerland

Site Status RECRUITING

University of Zurich, Department of Rheumatology

Zurich, , Switzerland

Site Status RECRUITING

The Universitiy of Leeds, Division of Rheumatic and Musculoskeletal Disease, St James's University Hospital

Leeds, , United Kingdom

Site Status RECRUITING

Royal Free Hospital, University College London

London, , United Kingdom

Site Status RECRUITING

Countries

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France Germany Hungary Italy Switzerland United Kingdom

Central Contacts

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Yannick Allanore, Prof.

Role: CONTACT

Jérome Avouac, Prof.

Role: CONTACT

Other Identifiers

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HEALTH-F5-2012-305495-OT4

Identifier Type: -

Identifier Source: org_study_id

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