Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan

Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study

Pulmonary Arterial Hypertension (PAH) in the setting of Idiopathic Pulmonary Fibrosis(IPF)is a risk factor for morbidity and mortality in the peri-lung transplant(LT) setting. Currently there is no significant data to support the use of pulmonary vasodilators for PAH in the setting of interstitial lung disease such as IPF. The majority of IPF patients have PAH either at rest or during exercise. The study hypothesis is that bosentan may improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH.

Study Overview

• Status: Withdrawn
• Conditions: Idiopathic Pulmonary Fibrosis; Pulmonary Arterial Hypertension
• Intervention / Treatment: Drug: bosentan

Detailed Description

The purpose of this study was to evaluate bosentan in the setting of exercise or resting pulmonary hypertension in patients with underlying pulmonary fibrosis.

• Study Type: Interventional
• Phase: Phase 4

Contacts and Locations

Study Locations

• United States
  • California
    • Los Angeles, California, United States, 90095-1690
      • David Geffen School of Medicine UCLA
    • Los Angeles, California, United States, 90095-1690
      • Departments of Pulmonary and Critical Care, Cardiothoracic Surgery and Infectious Diseases at David Geffen School of Medicine at UCLA

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Idiopathic Pulmonary Fibrosis referred for lung transplantation
• Minimum 50 meter 6 minute walk distance
• No significant underlying liver disease

Exclusion Criteria:

• Significant liver disease or cirrhosis
• non ambulatory
• previous adverse reaction/allergy to Bosentan

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: Randomized
• Interventional Model: Parallel Assignment
• Masking: None (Open Label)

Number of Arms

3

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: treatment with bosentan; patients with resting or exercise induced PAH receive bosentan in a randomized open label fashion	Drug: bosentan; 62.5mg orally bid for first month, followed by 125mg bid thereafter; Other Names: tracleer
No Intervention: PAH group with no therapy; patients with resting or exercise PAH get randomized to receive no specific therapy
No Intervention: No PAH and no therapy; patients with no evidence of either resting or exercise PAH receive no intervention but are followed until lung transplantation

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
6 minute walk distance	ATS Guideline 6MW distance before and after intervention	monthly assessement until date of lung transplantation

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
right heart catheterization hemodynamics	pulmonary hemodynamics	variable based on time between listing and actual lung transplantation
chemokine peripheral blood analysis	battery of chemokines analyzed from the peripheral blood	monthly

Collaborators and Investigators

• Sponsor: Rajan Saggar
• Collaborators: Actelion
• Investigators: Principal Investigator: Rajan Saggar, MD, University of California, Los Angeles

Study record dates

Study Major Dates

• Study Start (Actual): October 1, 2007
• Primary Completion (Anticipated): June 1, 2009
• Study Completion (Anticipated): December 1, 2009

Study Registration Dates

• First Submitted: February 19, 2008
• First Submitted That Met QC Criteria: February 19, 2008
• First Posted (Estimate): February 28, 2008

Study Record Updates

• Last Update Posted (Actual): March 6, 2018
• Last Update Submitted That Met QC Criteria: March 2, 2018
• Last Verified: March 1, 2018

More Information

Terms related to this study

• Keywords: idiopathic pulmonary fibrosis; pulmonary arterial hypertension; bosentan
• Additional Relevant MeSH Terms: Pathologic Processes; Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hypertension, Pulmonary; Fibrosis; Hypertension; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Molecular Mechanisms of Pharmacological Action; Antihypertensive Agents; Endothelin Receptor Antagonists; Bosentan
• Other Study ID Numbers: IPF/PAH

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: Yes