Chest
Original Research: Pulmonary Vascular DiseaseBosentan for Sarcoidosis-Associated Pulmonary Hypertension: A Double-Blind Placebo Controlled Randomized Trial
Section snippets
Materials and Methods
Patients aged 18 to 90 years with sarcoidosis defined by standard criteria16 were eligible for consideration. The patients were included if they had documented pulmonary hypertension with a PA mean pressure ≥ 25 mm Hg as measured by cardiac catheterization within 6 months of entry into the study. Pulmonary artery occlusion pressure (PAOP), left ventricular end-diastolic pressure, or both was < 15 mm Hg. Patients had to exhibit New York Heart Association (NYHA) functional class II or III
Results
Forty-three patients from five sites were enrolled in the study over a 4-year period, ending in September 2011. Figure 1 summarizes the outcomes for these patients. Four patients were never randomized because of withdrawn consent (two patients) or ineligibility (one each for liver dysfunction and NYHA functional class IV). Of the 39 randomized patients, 35 completed 16 weeks of therapy. Five patients declined repeat right-sided heart catheterization but continued in the study. One patient had a
Discussion
Based on case reports and series,12, 15, 21 we studied the effectiveness of bosentan in the treatment of SAPH. We found that 16 weeks of bosentan treatment was associated with a significant improvement in pulmonary hemodynamics as measured by both PA mean pressure and PVR. No improvement in pulmonary hemodynamics was found in the placebo group. The change in PA mean pressure was significantly different between the groups, and no significant changes in functional class, quality of life, or 6MWD
Acknowledgments
Author contributions: Dr Baughman had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Dr Baughman: contributed to the study design and execution, patient recruitment, and review of the final manuscript.
Dr Culver: contributed to the study design and execution, patient recruitment, and review of the final manuscript.
Dr Cordova: contributed to the study design and execution, patient recruitment, and review of
References (36)
- et al.
Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study
Lancet
(2001) - et al.
Updated clinical classification of pulmonary hypertension
J Am Coll Cardiol
(2009) - et al.
Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation
Chest
(2010) - et al.
Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension
Chest
(2005) - et al.
Predicting mortality in patients with sarcoidosis awaiting lung transplantation
Chest
(2003) - et al.
Treatment of sarcoidosis-associated pulmonary hypertension. A two-center experience
Chest
(2009) - et al.
Pulmonary hypertension in end-stage pulmonary sarcoidosis: therapeutic effect of sildenafil?
J Heart Lung Transplant
(2008) - et al.
Six-minute walk test and health status assessment in sarcoidosis
Chest
(2007) - et al.
Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial
Lancet
(2008) - et al.
Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial
J Am Coll Cardiol
(2008)
Acute pulmonary exacerbations of sarcoidosis
Chest
Bosentan decreases pulmonary vascular resistance and improves exercise capacity in acute hypoxia
Chest
Guidelines on diagnosis and treatment of pulmonary arterial hypertension
Eur Heart J
ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association
Circulation
Survival with first-line bosentan in patients with primary pulmonary hypertension
Eur Respir J
Clinical predictors of pulmonary hypertension in sarcoidosis
Eur Respir J
Inhaled iloprost for sarcoidosis associated pulmonary hypertension
Sarcoidosis Vasc Diffuse Lung Dis
Ambrisentan for sarcoidosis associated pulmonary hypertension
Sarcoidosis Vasc Diffuse Lung Dis
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Funding/Support: This study was supported by Actelion Pharmaceuticals US, Inc.
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