Treprostinil Therapy For Patients With Interstitial Lung Disease And Severe Pulmonary Arterial Hypertension

Pulmonary Arterial Hypertension Clinical Trial

Official title:

Using Either Intravenous (IV) or Subcutaneous (SQ) Treprostinil to Treat Pulmonary Hypertension Related to Underlying Interstitial Lung Disease

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT00705133
• Other study ID #: 07-11-087-01
• Status: Completed
• Phase: Phase 2
• Start date: July 2008
• Est. completion date: April 2011

Study information

• Verified date: September 2020
• Source: University of California, Los Angeles
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

Our hypothesis is that IV or SQ Treprostinil can improve 6 minute walk distance, hemodynamics and quality of life in patients with interstitial lung disease and severe secondary pulmonary arterial hypertension.

Description:

Patients with pulmonary hypertension (PH) complicating pulmonary fibrosis are at increased risk of death. There are no therapies proven to be effective in this population, targeting the pulmonary hypertension. The purpose of this study is to evaluate parenteral treprostinil in an open-label fashion in patients with pulmonary fibrosis and an advanced PH phenotype.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 15
• Est. completion date: April 2011
• Est. primary completion date: January 2011
• Accepts healthy volunteers: No
• Gender: All
• Age group: N/A and older

Eligibility

Inclusion Criteria:

Eligible subjects must have IPF and severe pulmonary arterial hypertension (PAH) documented on standard of care right-heart catheterization (RHC) and planned to receive therapy with treprostinil as recommended by the treating physician.

1. All subjects must have high resolution CT scan (HRCT) diagnostic of IPF (performed as part of standard of care evaluation) or if available, biopsy proven histological usual interstitial pneumonia (UIP).

2. Severe pulmonary arterial hypertension defined as a resting mean pulmonary artery pressure (mPAP) > 35 mm Hg; AND pulmonary vascular resistance (PVR) > 3 woods-units; AND pulmonary capillary wedge pressure (PCWP) < 18 mm Hg by right-heart catheterization (RHC) performed as part of standard of care evaluation.

3. All subjects must be planned to receive treprostinil therapy as recommended by their treating physician.

Exclusion Criteria:

1. Acute or chronic impairment other than dyspnea (e.g. angina pectoris, intermittent claudication) limiting the ability to perform standard of care six-minute walk tests (6MWT).

2. Six-minute walk distance (6MWD) < 50 meters at screening or baseline standard of care evaluations

3. Standard of care pulmonary function test (PFT) showing forced expiratory volume in one second (FEV1)/ forced vital capacity (FVC) ratio < 0.65

4. Standard of care pulmonary function test (PFT) showing a residual volume >120% predicted

5. Standard of care high-resolution chest computed tomography (HRCT) showing emphysema extent > 30%

6. Any investigational therapy as part of a clinical trial for any indication with 30 days before screening

7. Change in dose of treatment for IPF - investigational agent (gamma interferon-1b, pirfenidone, etanercept, and any other investigational agent intended to treat IPF), corticosteroids, or cytotoxic agents, within 30 days before screening. That is, subjects can be on any of these agents provided the dose is stable for at least 30 days prior to enrollment.

8. Current treatment for pulmonary hypertension with other prostaglandins (epoprostenol or iloprost)

9. Change in dose of treatment for PAH - (bosentan, sitaxsentan, ambrisentan, tadalafil, sildenafil, vardenafil, calcium channel blockers, nitrates, digitalis), within 30 days before screening. That is, subjects can be on any of these agents provided the dose is stable for at least 30 days prior to enrollment

10. Pulmonary rehabilitation initiated within 30 days of baseline.

Related Conditions & MeSH terms

• Familial Primary Pulmonary Hypertension
• Fibrosis
• Hypertension
• Idiopathic Pulmonary Fibrosis
• Interstitial Lung Disease
• Lung Diseases
• Lung Diseases, Interstitial
• Pulmonary Arterial Hypertension
• Pulmonary Fibrosis

Intervention

Drug:
• Treprostinil
For both SQ and IV routes, treprostinil will be started in the hospital at 1ng/kg/min and titrated up by 1ng/kg/min every 1-3 days as tolerated

Locations

Country	Name	City	State
United States	David Geffen School of Medicine, UCLA	Los Angeles	California

Sponsors (2)

Lead Sponsor	Collaborator
Rajan Saggar	United Therapeutics

Country where clinical trial is conducted

United States, 

References & Publications (1)

Saggar R, Khanna D, Vaidya A, Derhovanessian A, Maranian P, Duffy E, Belperio JA, Weigt SS, Dua S, Shapiro SS, Goldin JG, Abtin F, Lynch JP 3rd, Ross DJ, Forfia PR, Saggar R. Changes in right heart haemodynamics and echocardiographic function in an advanc — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	6 Minute Walk Distance	American Thoracic Society (ATS) Practice Guideline based 6-minute walk (6MW) distance	3 months
Secondary	Pulmonary Vascular Resistance	repeat right heart catheterization	3 months
Secondary	SF-36 Quality of Life	SF-36 physical component summary (PCS) which is one component of the SF-36 survey. The health assessment questionnaire Short Form 36 Version 2.0 (SF-36 V2 ) determines participants' overall quality of life by assessing 1) limitations in physical functioning due to health problems; 2) limitations in usual role because of physical health problems; 3) bodily pain; 4) general health perceptions; 5) vitality; 6) limitations in social functioning because of physical or emotional problems; 7) limitations in usual role due to emotional problems; and 8) general mental health. Items 1-4 primarily contribute to the physical component summary score (PCS) of the SF-36. Scores on each item are summed and averaged (range = 0 "worst"-100 "best").	3 months
Secondary	Brain Natriuretic Peptide	brain natriuretic peptide (BNP) is a measure of right ventricular distension and/or stress due to pressure or volume overload	3 months