期刊
CLINICS IN CHEST MEDICINE
卷 36, 期 4, 页码 703-+出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.ccm.2015.08.011
关键词
Bosentan; Sarcoidosis; Pulmonary hypertension; Dyspnea
资金
- Actelion
- Gilead
- United Therapeutics
- Bayer
Pulmonary hypertension is a complication of sarcoidosis leading to dyspnea and associated with increased morbidity and mortality. Sarcoidosis-associated pulmonary hypertension (SAPH) can be due to several factors, including vascular involvement by the granulomatous inflammation, compression of the pulmonary arteries by adenopathy, fibrotic changes within the lung, and left ventricular diastolic dysfunction. Several case series have suggested that some patients with SAPH benefit from specific therapy for pulmonary hypertension. A randomized, placebo-controlled trial found 16 weeks' bosentan therapy to be associated with significant improvement in pulmonary artery pressure. Future studies may better define who would respond to treatment of pulmonary hypertension.
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