Interstitial Lung Disease, Pulmonary Hypertension
Conditions
Keywords
ILD, PH, PH-ILD
Brief summary
Study GMS-PH-001 is a multicenter, open-label, non-randomized study to prospectively evaluate screening strategies of pulmonary hypertension (PH) in patients with interstitial lung disease (ILD).
Detailed description
In this study, subjects will undergo a broad range of clinical assessments that are potentially associated with PH. Study data will be used to identify and weigh specific clinical parameters based on their prognostic significance for right heart catheterization (RHC)-confirmed PH. There is no study drug under investigation in this study. The study consists of 2 study visits: a Screening Visit and Study Visit 1. Clinical assessments include pulmonary function tests (PFTs); high resolution computed tomography (HRCT); physical examination; 6-Minute Walk Test; blood draw for clinical laboratory parameters, plasma brain natriuretic peptide (BNP) concentration and plasma N-terminal pro-BNP (NT-proBNP) concentration; echocardiography; University of California San Diego Shortness of Breath Questionnaire (UCSD SOBQ); King's Brief Interstitial Lung Disease Questionnaire (K-BILD); 36-Item Short Form Survey (SF-36); Pulmonary Hypertension Functional Classification Self Report (PH-FC-SR); Investigator's Suspicion of PH Questionnaire; adverse event (AE) monitoring; and RHC. As the primary objective of this study is to collect a broad range of clinical parameters in patients with ILD, it is expected that numerous exploratory and post-hoc analyses will be performed to identify and weigh specific parameters based on their prognostic significance for PH in this patient population with the goal of developing a screening algorithm for PH in patients with ILD.
Interventions
PROCEDURERight heart catheterization (RHC)
RHC to evaluate pulmonary hemodynamics
Sponsors
United Therapeutics
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
1. Patient gives voluntary written informed consent to participate in the study and are capable of reading and understanding questionnaires or forms in English or Spanish. 2. Patients with a diagnosis of ILD based on computed tomography imaging, including: 1. Idiopathic interstitial pneumonia, including idiopathic pulmonary fibrosis 2. Connective tissue disease-associated ILD with forced vital capacity (FVC) \<70% 3. Hypersensitivity pneumonitis 4. Scleroderma-related ILD 5. Autoimmune ILD 6. Nonspecific interstitial pneumonia 7. Occupational lung disease 8. Combined pulmonary fibrosis and emphysema with mild emphysema on lung imaging as determined by the Investigator 3. Patients must have at least 2 signs or symptoms suggestive of PH, as specified by the study protocol.
Exclusion criteria
1. Prior RHC with mPAP \>20 mmHg. 2. Currently on a Food and Drug Administration (FDA)-approved pulmonary arterial hypertension (PAH) medication for the treatment of PAH. 3. Diagnosed with chronic obstructive pulmonary disease (CPFE with mild emphysema notwithstanding). 4. Uncontrolled or untreated moderate to severe sleep apnea as determined by the patient's physician. 5. Pulmonary embolism within the past 3 months. 6. History of ischemic heart disease or left-sided myocardial dysfunction within 12 months of Screening, defined as left ventricular ejection fraction \<40% or pulmonary capillary wedge pressure \>15 mmHg. 7. Any other clinical features that, in the opinion of the Investigator, might adversely affect interpretation of study data or study safety, or make the patient unsuitable for RHC or HRCT (e.g., pregnancy).
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Percentage of patients with PH as indicated by RHC | Through study completion, approximately 3 weeks. | Mean pulmonary artery pressure (mPAP) \>20 mmHg with pulmonary artery wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) \>2 WU. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Percentage of patients with severe PH as indicated by RHC | Through study completion, approximately 3 weeks. | mPAP \>20 mmHg with PAWP ≤15 mmHg and PVR \>5 WU. |
Countries
United States
Outcome results
None listed