Patients with advanced sarcoidosis often develop pulmonary hypertension. Pulmonary hypertension is a condition where the right side of the heart has to push the blood though the lungs at a higher pressure than normal. Since this pressure is higher, it is harder for the heart to pump the blood through the lungs to the left side of the body. If the blood can not get through the lungs, it can not get pumped through the rest of the body. This leads to weakness and shortness of breath. This type of hypertension does not usually respond to regular blood pressure medicines. The purpose of this study is to determine if bosentan (Tracleer) will help sarcoidosis associated pulmonary hypertension.
Patients will be randomized in 2:1 manner to receive either bosentan or placebo for 16 weeks. After 16 weeks, there will be an additional 32 weeks of an open label extension.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
TRIPLE
Enrollment
43
Mount Sinai
New York, New York, United States
University of Cincinnati
Cincinnati, Ohio, United States
Cleveland Clinic
Cleveland, Ohio, United States
University of Pittsburgh
Pittsburgh, Pennsylvania, United States
Improvement in six minute walk distance
Time frame: 16 weeks
Change in pulmonary hemodynamics
Time frame: 16 weeks
Improvement in quality of life with therapy
Time frame: 48 weeks
Safety of treatment
Time frame: 48 weeks
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