Clinical trial NCT06388421: DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)
Official title: DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Pulmonary Hypertension Due to Lung Diseases and Hypoxia
Pulmonary Hypertension
Interstitial Lung Disease
Keywords: pulmonary hypertension
interstitial lung disease
pulmonary hypertension associated with interstitial lung disease
Lead sponsor: United Therapeutics
Sponsor class: INDUSTRY
First posted: 2024-04-29
Enrollment: 1000
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patients with a diagnosis of pulmonary hypertension associated with interstitial lung disease. This includes patients who are and aren't receiving inhaled treprostinil, excluding patients receiving Yutrepia™ at Baseline.

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

Detailed 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.

Eligibility criteria: 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.

Primary outcomes:
measure: Baseline characteristics as described by demographics, medical history, ILD history, PH history, family history (comorbidities), and diagnostic right heart catheterization values
timeFrame: Baseline
Secondary outcomes:
measure: Changes in clinical features relating to patients' interstitial lung disease and pulmonary hypertension
timeFrame: Followed prospectively for a maximum of 5 years from date of enrollment into the registry
Other outcomes:
Locations:
city: Birmingham
country: United States
facility: University of Alabama at Birmingham
state: Alabama
city: Naples
country: United States
facility: NCH Research Institute
state: Florida
city: Sarasota
country: United States
facility: Intercoastal Medical Group
state: Florida
city: Indianapolis
country: United States
facility: Ascension St. Vincent
state: Indiana
city: Kansas City
country: United States
facility: University of Kansas Medical Center
state: Kansas
city: Louisville
country: United States
facility: Norton Pulmonary Specialists
state: Kentucky
city: New Orleans
country: United States
facility: University Medical Center - New Orleans
state: Louisiana
city: Albuquerque
country: United States
facility: University of New Mexico Health Sciences Center
state: New Mexico
city: New Hyde Park
country: United States
facility: Northwell Health
state: New York
city: Bend
country: United States
facility: Summit Health
state: Oregon
city: Portland
country: United States
facility: Legacy Research Institute
state: Oregon
city: Portland
country: United States
facility: Oregon Health & Science University
state: Oregon
city: Anderson
country: United States
facility: AnMed Health
state: South Carolina
city: Dallas
country: United States
facility: Baylor University Medical Center
state: Texas
city: Richmond
country: United States
facility: Pulmonary Associates of Richmond, Inc.
state: Virginia
city: Huntington
country: United States
facility: Marshall Health
state: West Virginia
Study officials:
References: