Development and Prevention of Pulmonary Hypertension in Systemic Sclerosis

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

Sponsors & Collaborators

• European Union

  collaborator OTHER

• University of Giessen

  collaborator OTHER

• University of Zurich

  collaborator OTHER

• University of Florence

  collaborator OTHER

• University of Campania Luigi Vanvitelli

  collaborator OTHER

• University of Basel

  collaborator OTHER

• University College, London

  collaborator OTHER

• Charite University, Berlin, Germany

  collaborator OTHER

• University of Pecs

  collaborator OTHER

• University of Leeds

  collaborator OTHER

• Schoen Klinik Hamburg Eilbek

  collaborator OTHER

• University of Paris 5 - Rene Descartes

  lead OTHER

Principal Investigators

• Yannick Allanore, Prof. · Université Paris Descartes, Hôpital Cochin, Service de Rhumatologie A & INSERM 1016

• Ulf Müller-Ladner, Prof. · Justus-Liebig-University Gießen, Kerckhoff Clinic, Departement of Rheumatology and Clinical Immunology

Eligibility

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2013-04-30

Primary Completion

2017-06-30

Completion

2017-06-30

Countries

• France
• Germany
• Hungary
• Italy
• Switzerland
• United Kingdom

Study Locations