Ambrisentan (Letairis) for Sarcoidosis Associated Pulmonary Hypertension

Medical University of South Carolina (MUSC)

Status and phase: Completed; Phase 3; Phase 2

Conditions: Sarcoidosis; Pulmonary Hypertension

Treatments: Drug: Ambrisentan

Study type: Interventional

Funder types: Other; Industry

Identifiers

NCT00851929

17747

Details and patient eligibility

About

Hypothesis: Ambrisentan (Letairis ®) is safe and effective in treating pulmonary hypertension in patients with Sarcoidosis

Full description

Primary Endpoint: Change in 6 minute walk distance.

Enrollment: 16 patients

Sex: All

Ages: 18 to 99 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Biopsy proven sarcoidosis
• Mean pulmonary artery pressure > 25 mmHg at rest and greater than 30 mmHg with exercise by right heart catheterization within 1 year prior to entry into study
• Pulmonary capillary wedge pressure ≤ 15 mmHg
• PVR values >3.0 Woods units
• Forced vital capacity (FVC) >40%
• WHO functional class II or III
• Stable sarcoidosis treatment regimen for three months prior to entry into study
• 6 minute walk distance between 150-450 meters
• Stable dose of antihypertensive medications
• On no other medication to treat PAH (sildenafil, tadalafil, vardenafil, treprostinil, epoprostenol, iloprost, bosentan, sitaxsentan) within one month prior to enrollment and during duration of the study
• Non-pregnant females

Exclusion criteria

• Exercise limitation related to a non-cardiopulmonary reason (e.g. arthritis)
• Severe systemic hypertension > 170/95
• Patients with congestive heart failure (left ventricular dysfunction) or primary right ventricular dysfunction
• Anticipation by the investigator for escalation in sarcoidosis treatment during the course of the study
• Pulmonary hypertension related to etiology other than sarcoidosis (i.e. HIV, scleroderma, etc.)
• Use within 1 month of an endothelin receptor antagonists (bosentan, sitaxsentan).
• WHO functional class IV status
• Patients with significant left ventricular dysfunction
• Significant liver dysfunction not due to sarcoidosis.
• Patients with severe other organ disease felt by investigators to impact on survival during the course of the study.

Trial design

Primary purpose: Treatment

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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16 participants in 1 patient group

sarcoidosis associated pulmonary hypertension

Experimental group

Description:

sarcoidosis associated pulmonary hypertension

Treatment:

Drug: Ambrisentan