Ambrisentan (Letairis) for Sarcoidosis Associated Pulmonary Hypertension

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

Detailed Description
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Primary Endpoint: Change in 6 minute walk distance.

Official Title
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Ambrisentan (Letairis) for Sarcoidosis Associated Pulmonary Hypertension

Conditions
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Sarcoidosis, Pulmonary Hypertension

Intervention / Treatment
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* Drug: Ambrisentan


Participation Criteria
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Eligibility Criteria
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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.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 99 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: sarcoidosis associated pulmonary hypertension<br>sarcoidosis associated pulmonary hypertension | Drug: Ambrisentan<br>* ambrisentan 5 mg/day for month month, then 10 mg/day for 3 additional months<br>* Other names: Letairis;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in 6 minute walk distance. |  | 4 months of therapy | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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sarcoidosis, pulmonary hypertension, dyspnea, sarcoidosis associated pulmonary hypertension