Development and Prevention of Pulmonary Hypertension in Systemic Sclerosis

NCT01840748 | Unknown

• 1 other identifier: HEALTH-F5-2012-305495-OT4
• Study Type: observational
• Target: 960
• Locations: 6 countries
• Sites: 11

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.

Conditions

Systemic Sclerosis; Pulmonary Hypertension

Outcome Measures

Primary Outcomes (1)

• The number of patients with pulmonary hypertension at 2 years

  2 years

Secondary Outcomes (1)

• Time to development of precapillary pulmonary hypertension

  2 years

Study Arms (3)

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

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

The study population are adult and juvenile systemic sclerosis patients from the EUSTAR cohort (MEDSonline database) and the juvenile systemic sclerosis working group cohort

You may qualify if:

• 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

Sponsors & Collaborators

• University of Paris 5 - Rene Descartes: lead
• European Union: collaborator
• University of Giessen: collaborator
• University of Zurich: collaborator
• University of Florence: collaborator
• University of Campania Luigi Vanvitelli: collaborator
• University of Basel: collaborator
• University College, London: collaborator
• Charite University, Berlin, Germany: collaborator
• University of Pecs: collaborator
• University of Leeds: collaborator
• Schoen Klinik Hamburg Eilbek: collaborator

Study Sites (11)

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

Paris, 75014, France

RECRUITING

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

Bad Nauheim, 61231, Germany

RECRUITING

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

Berlin, 10117, Germany

RECRUITING

Centre for Pediatric Rheumatology, Klinikum Eilbek

Hamburg, 22081, Germany

RECRUITING

Pecsi Tudomanyegyetem - University of Pecs

Pécs, H-7622, Hungary

RECRUITING

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

Florence, 50139, Italy

RECRUITING

Policlinico, Via Pansini

Napoli-Italia, 5-80131, Italy

RECRUITING

Felix-Platter Spital, University of Basel

Basel, CH 4012 Basel, Switzerland

RECRUITING

University of Zurich, Department of Rheumatology

Zurich, 8006, Switzerland

RECRUITING

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

Leeds, LS9 7TF, United Kingdom

RECRUITING

Royal Free Hospital, University College London

London, NW3 2QG, United Kingdom

RECRUITING

MeSH Terms

Conditions

Scleroderma, Systemic; Hypertension, Pulmonary

Condition Hierarchy (Ancestors)

Connective Tissue Diseases; Skin and Connective Tissue Diseases; Skin Diseases; Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Study Officials

• 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

  STUDY CHAIR

Central Study Contacts

Yannick Allanore, Prof.

CONTACT

yannick.allanore@cch.aphp.fr

Jérome Avouac, Prof.

CONTACT

javouac@yahoo.fr

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 2 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Prof. Yannik Allanore

Study Record Dates

• First Submitted: April 19, 2013
• First Posted: April 26, 2013
• Study Start: April 1, 2013
• Primary Completion: June 1, 2017
• Study Completion: June 1, 2017
• Last Updated: April 26, 2013

Record last verified: 2013-04

Locations

FR (1); HU (1); CH (2); GB (2); IT (2); DE (3)