DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)

Pulmonary Hypertension Clinical Trial

Official title:

DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT06388421
• Other study ID #: GMS-PH-401
• Status: Not yet recruiting
• Start date: August 1, 2024
• Est. completion date: November 30, 2030

Study information

• Verified date: May 2024
• Source: United Therapeutics
• Contact: United Therapeutics Global Medical Information
• Phone: 919-485-8350
• Email: clinicaltrials[@]unither.com
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

This is a prospective, non-interventional, multicenter, registry of patients with pulmonary associated with interstitial lung disease (PH-ILD).

Description:

The purpose of this study is to observe and capture demographic characteristics, treatment patterns, and clinical outcomes of interest for patients with PH-ILD to further clinical understanding of the epidemiological landscape and outcomes of the disease and treatment. In this registry, patients will be enrolled into 1 of 3 cohorts. Cohort 1 will include approximately 500 patients who are not receiving inhaled treprostinil at time of study enrollment. Cohort 2 will include approximately 150 patients who are newly initiated on Tyvaso/Tyvaso DPI. Cohort 3 will include approximately 350 patients who have been receiving Tyvaso/Tyvaso DPI for >60 days. As part of the registry, assessments will be completed for data collection in 6- or 12 month intervals, unless the data are already available via standard of care. This patient registry will follow patients for up to 5 years after enrollment. Assessments include pulmonary function tests (PFTs) including diffusing capacity of the lungs for carbon monoxide (DLCO); high resolution computed tomography (HRCT); vital signs; 6-Minute Walk Test; blood draw for plasma N-terminal pro-BNP (NT-proBNP) concentration, genetics analysis, biomarker analysis; echocardiography; EuroQoL 5 Dimension 5 Level Questionnaire (EQ-5D-5L); King's Brief Interstitial Lung Disease Questionnaire (K-BILD); Living with Pulmonary Fibrosis Questionnaire (L-PF); Therapy Administration Questionnaire; healthcare resource utilization; current medications and rehabilitation; WHO functional class; transplant status; survival data; and RHC.

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 1000
• Est. completion date: November 30, 2030
• Est. primary completion date: August 1, 2030
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

1. Adults aged 18 years or older

2. Diagnosis of ILD by traditional or HRCT determined by the site/institution that conducted the HRCT

3. Patients with connective tissue disease must have a baseline forced vital capacity of <70%

4. RHC confirmed PH (mean pulmonary artery pressure >20 mmHg, pulmonary arterial wedge pressure =15 mmHg, pulmonary vascular resistance >2 WU).

5. For patients to be eligible for Cohort 1, they must not be receiving inhaled treprostinil at Baseline.

6. For patients to be eligible for Cohort 2, they must have initiated Tyvaso/Tyvaso DPI

at 1 of the following time points:

1. Baseline

2. =60 days prior to Baseline

7. For patients to be eligible for Cohort 3, they must be receiving Tyvaso/Tyvaso DPI at Baseline and for >60 days prior to Baseline

8. Co-enrollment in other observational or interventional studies is permitted

9. Patient is willing and able to provide informed consent and complete surveys/questionnaires in English or Spanish

Exclusion Criteria:

1. Confirmed diagnosis of Group 1, 2, 4, or 5 PH

2. Confirmed diagnosis of Group 3 PH related to chronic obstructive pulmonary disease or conditions that cause hypoxemia, such as untreated or inadequately treated obstructive sleep apnea and alveolar hypoventilation disorders

3. Patients receiving Yutrepia (inhaled treprostinil) at Baseline.

Related Conditions & MeSH terms

• Hypertension
• Hypertension, Pulmonary
• Hypoxia
• Interstitial Lung Disease
• Lung Diseases
• Lung Diseases, Interstitial
• Pulmonary Hypertension
• Pulmonary Hypertension Due to Lung Diseases and Hypoxia

Intervention

Other:
• Prospective study assessments
Patients will complete prospective assessments at 6- and 12-month intervals for up to 5 years. There will be 1 check in at the first 3 months of enrollment. Follow up visits will include a ±45 day visit window. If an assessment is performed within 45 days of the visit window as part of the patient's standard of care, those values will be used in place of a prospective assessment.

Locations

Country	Name	City	State
n/a

Sponsors (1)

Lead Sponsor	Collaborator
United Therapeutics

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Baseline characteristics as described by demographics, medical history, ILD history, PH history, family history (comorbidities), and diagnostic right heart catheterization values		Baseline
Secondary	Changes in clinical features relating to patients' interstitial lung disease and pulmonary hypertension		Followed prospectively for a maximum of 5 years from date of enrollment into the registry