NCT03726398

CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated ILD and PH

CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated Interstitial Lung DiseasE and Pulmonary Hypertension (PH): The CRuSADE PH Study

• Status: Withdrawn
• Phase: Phase 2 / Phase 3
• Study type: Interventional
• Enrollment: 0 (actual)
• Sponsor: Franz Rischard, DO · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Not accepted

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.

Detailed 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.

Conditions

• Interstitial Lung Disease
• Scleroderma
• Pulmonary Hypertension

Interventions

Type	Name	Description
DRUG	Opsumit 10 Mg Tablet	Oral tablet taken once daily

Timeline

• Start date: 2018-09-01
• Primary completion: 2020-08-03
• Completion: 2020-08-03
• First posted: 2018-10-31
• Last updated: 2025-02-20

Locations

1 site across 1 country: United States

Regulatory

• FDA-regulated drug study