Pulmonary Hypertension Clinical Trial
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.
n/a
Observational Model: Cohort, Time Perspective: Prospective
| Status | Clinical Trial | Phase | |
|---|---|---|---|
| Withdrawn |
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