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

Rajan Saggar

Status and phase: Withdrawn; Phase 4

Conditions: Idiopathic Pulmonary Fibrosis; Pulmonary Arterial Hypertension

Treatments: Drug: bosentan

Study type: Interventional

Funder types: Other; Industry

Identifiers

NCT00625469

IPF/PAH

Details and patient eligibility

About

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.

Full 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.

Sex: All

Volunteers: No Healthy Volunteers

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

Trial design

Primary purpose: Treatment

Allocation: Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

---

0 participants in 3 patient groups

treatment with bosentan

Experimental group

Description:

patients with resting or exercise induced PAH receive bosentan in a randomized open label fashion

Treatment:

Drug: bosentan

PAH group with no therapy

No Intervention group

Description:

patients with resting or exercise PAH get randomized to receive no specific therapy

No PAH and no therapy

No Intervention group

Description:

patients with no evidence of either resting or exercise PAH receive no intervention but are followed until lung transplantation