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Clinical Trial Summary

Patients with interstitial lung disease (ILD) and scleroderma who develop pulmonary hypertension (PH) do not fit well into the current classification system and treatments for pulmonary hypertension. This study aims to better understand patients with ILD-PH and scleroderma and to determine if treatment with Macitentan is beneficial.


Clinical Trial Description

The investigators aim to use pressure-volume loop derived right ventriculo-vascular coupling, pulmonary impedance, and invasive cardiopulmonary exercise testing (CPET) to:

1. Comprehensively phenotype patients with scleroderma ILD-PH and pulmonary vascular exercise limitation (PVL) relative to scleroderma ILD-PH without PVL.

2. Compare the efficacy of chronic Macitentan therapy in improving 1) right ventricular hemodynamics 2) exercise capacity and 3) symptoms in scleroderma ILD-PH patients with and without PVL. ;


Study Design


Related Conditions & MeSH terms


NCT number NCT03726398
Study type Interventional
Source University of Arizona
Contact Valerie Bloss, MS
Phone 520-626-8000
Email vbloss@email.arizona.edu
Status Recruiting
Phase Phase 2/Phase 3
Start date September 1, 2018
Completion date December 1, 2020

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