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

Primary Purpose

Pulmonary Arterial Hypertension, Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis

Status

Completed

Phase

Phase 2

Locations

United States

Study Type

Interventional

Intervention

Treprostinil

About this trial

This is an interventional treatment trial for Pulmonary Arterial Hypertension focused on measuring pulmonary hypertension, pulmonary fibrosis, interstitial lung disease

Eligibility Criteria

undefined - undefined (Child, Adult, Older Adult)All SexesDoes not accept healthy volunteers

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.

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

Exclusion Criteria:

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).
Six-minute walk distance (6MWD) < 50 meters at screening or baseline standard of care evaluations
Standard of care pulmonary function test (PFT) showing forced expiratory volume in one second (FEV1)/ forced vital capacity (FVC) ratio < 0.65
Standard of care pulmonary function test (PFT) showing a residual volume >120% predicted
Standard of care high-resolution chest computed tomography (HRCT) showing emphysema extent > 30%
Any investigational therapy as part of a clinical trial for any indication with 30 days before screening
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.
Current treatment for pulmonary hypertension with other prostaglandins (epoprostenol or iloprost)
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
Pulmonary rehabilitation initiated within 30 days of baseline.

Sites / Locations

David Geffen School of Medicine, UCLA

Arms of the Study

Arm 1

Arm Type

Experimental

Arm Label

Treprostinil-treated

Arm Description

Patients with pulmonary fibrosis with an advanced pulmonary hypertension phenotype will be treated with parenteral treprostinil in an open-label fashion

Outcomes

Primary Outcome Measures

6 Minute Walk Distance

American Thoracic Society (ATS) Practice Guideline based 6-minute walk (6MW) distance

Secondary Outcome Measures

Pulmonary Vascular Resistance

repeat right heart catheterization

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").

Brain Natriuretic Peptide

brain natriuretic peptide (BNP) is a measure of right ventricular distension and/or stress due to pressure or volume overload

Full Information

NCT ID

NCT00705133

First Posted

June 23, 2008

Last Updated

September 7, 2020

Sponsor

Rajan Saggar

Collaborators

United Therapeutics

1. Study Identification

Unique Protocol Identification Number

NCT00705133

Brief Title

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

Official Title

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

Study Type

Interventional

2. Study Status

Record Verification Date

September 2020

Overall Recruitment Status

Completed

Study Start Date

July 2008 (Actual)

Primary Completion Date

January 2011 (Actual)

Study Completion Date

April 2011 (Actual)

3. Sponsor/Collaborators

Responsible Party, by Official Title

Sponsor-Investigator

Name of the Sponsor

Rajan Saggar

Collaborators

United Therapeutics

4. Oversight

Data Monitoring Committee

5. Study Description

Brief 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.

Detailed 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.

6. Conditions and Keywords

Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study

Pulmonary Arterial Hypertension, Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis

Keywords

pulmonary hypertension, pulmonary fibrosis, interstitial lung disease

7. Study Design

Primary Purpose

Treatment

Study Phase

Phase 2

Interventional Study Model

Single Group Assignment

Masking

None (Open Label)

Allocation

N/A

Enrollment

15 (Actual)

8. Arms, Groups, and Interventions

Arm Title

Treprostinil-treated

Arm Type

Experimental

Arm Description

Patients with pulmonary fibrosis with an advanced pulmonary hypertension phenotype will be treated with parenteral treprostinil in an open-label fashion

Intervention Type

Drug

Intervention Name(s)

Treprostinil

Other Intervention Name(s)

remodulin

Intervention Description

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

Primary Outcome Measure Information:

Title

6 Minute Walk Distance

Description

American Thoracic Society (ATS) Practice Guideline based 6-minute walk (6MW) distance

Time Frame

3 months

Secondary Outcome Measure Information:

Title

Pulmonary Vascular Resistance

Description

repeat right heart catheterization

Time Frame

3 months

Title

SF-36 Quality of Life

Description

Time Frame

3 months

Title

Brain Natriuretic Peptide

Description

brain natriuretic peptide (BNP) is a measure of right ventricular distension and/or stress due to pressure or volume overload

Time Frame

3 months

10. Eligibility

Sex

All

Accepts Healthy Volunteers

Eligibility Criteria

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. 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). 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. All subjects must be planned to receive treprostinil therapy as recommended by their treating physician. Exclusion Criteria: 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). Six-minute walk distance (6MWD) < 50 meters at screening or baseline standard of care evaluations Standard of care pulmonary function test (PFT) showing forced expiratory volume in one second (FEV1)/ forced vital capacity (FVC) ratio < 0.65 Standard of care pulmonary function test (PFT) showing a residual volume >120% predicted Standard of care high-resolution chest computed tomography (HRCT) showing emphysema extent > 30% Any investigational therapy as part of a clinical trial for any indication with 30 days before screening 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. Current treatment for pulmonary hypertension with other prostaglandins (epoprostenol or iloprost) 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 Pulmonary rehabilitation initiated within 30 days of baseline.

Overall Study Officials:

First Name & Middle Initial & Last Name & Degree

Rajan Saggar, MD

Organizational Affiliation

David Geffen School of Medicine, UCLA

Official's Role

Principal Investigator

First Name & Middle Initial & Last Name & Degree

David Zisman, MD

Organizational Affiliation

David Geffen School of Medicine, UCLA

Official's Role

Principal Investigator

Facility Information:

Facility Name

David Geffen School of Medicine, UCLA

City

Los Angeles

State/Province

California

ZIP/Postal Code

90095

Country

United States

12. IPD Sharing Statement

Plan to Share IPD

Citations:

PubMed Identifier

24431095

Citation

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 advanced phenotype of pulmonary hypertension and right heart dysfunction associated with pulmonary fibrosis. Thorax. 2014 Feb;69(2):123-9. doi: 10.1136/thoraxjnl-2013-204150.

Results Reference

result

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

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