title: Pulmonary Hypertension in Sarcoidosis.
abstract: 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.
authors.affiliation: Department of Medicine, University of Cincinnati, 1001 Holmes Eden Avenue, Cincinnati, OH 45220, USA. Electronic address: bob.baughman@uc.edu.
authors.forename: Robert P
authors.initials: RP
authors.lastname: Baughman
authors.affiliation: Ohio Heart and Cardiovascular Center, Christ Hospital, Auburn Avenue, Cincinnati, OH 45219, USA.
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Engel
authors.affiliation: Advanced Lung Disease, INOVA Medical Care, 3300 Gallows Road, Falls Church, VA 22042, USA.
authors.forename: Steven
authors.initials: S
authors.lastname: Nathan
mesh_terms: D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008168:Lung / Q000503:physiopathology*; D012507:Sarcoidosis / Q000150:complications*
keywords: Bosentan; Dyspnea; Pulmonary hypertension; Sarcoidosis