title: Bosentan for sarcoidosis-associated pulmonary hypertension: a double-blind placebo controlled randomized trial.
abstract: BACKGROUND
  Sarcoidosis-associated pulmonary hypertension (SAPH) is a common problem in patients with persistent dyspneic sarcoidosis. The objective of this study was to determine the effect of bosentan therapy on pulmonary arterial hemodynamics in patients with SAPH.
  
  
  METHODS
  This 16-week study was a double-blind, placebo-controlled trial of either bosentan or placebo in patients with SAPH confirmed by right-sided heart catheterization. Patients were enrolled from multiple academic centers specializing in sarcoidosis care. They were stable on sarcoidosis therapy and were receiving no therapy for pulmonary hypertension. The cohort was randomized two to one to receive bosentan at a maximal dose of 125 mg or placebo bid for 16 weeks. Pulmonary function studies, 6-min walk test, and right-sided heart hemodynamics, including pulmonary artery mean pressure and pulmonary vascular resistance (PVR), were performed before and after 16 weeks of therapy.
  
  
  RESULTS
  Thirty-five patients completed 16 weeks of therapy (23 treated with bosentan, 12 with placebo). For those treated with bosentan, repeat hemodynamic studies at 16 weeks demonstrated a significant mean±SD fall in PA mean pressure (-4±6.6 mm Hg, P=.0105) and PVR (-1.7±2.75 Wood units, P=.0104). For the patients treated with placebo, there was no significant change in either PA mean pressure (1±3.7 mm Hg, P>.05) or PVR (0.1±1.42 Wood units, P>.05). There was no significant change in 6-min walk distance for either group. Two patients treated with bosentan required an increase of supplemental oxygen by >2 L after 16 weeks of therapy.
  
  
  CONCLUSIONS
  This study demonstrated that bosentan significantly improved pulmonary hemodynamics in patients with SAPH.
  
  
  BACKGROUND
  ClinicalTrials.gov; No: NCT00581607; URL: www.clinicaltrials.gov.
authors.affiliation: Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH. Electronic address: bob.baughman@uc.edu.
authors.forename: Robert P
authors.initials: RP
authors.lastname: Baughman
authors.affiliation: Cleveland Clinic Foundation, Cleveland, OH.
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Culver
authors.affiliation: Department of Internal Medicine, Temple University, Philadelphia, PA.
authors.forename: Francis C
authors.initials: FC
authors.lastname: Cordova
authors.affiliation: Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY.
authors.forename: Maria
authors.initials: M
authors.lastname: Padilla
authors.affiliation: Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA.
authors.forename: Kevin F
authors.initials: KF
authors.lastname: Gibson
authors.affiliation: Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH.
authors.forename: Elyse E
authors.initials: EE
authors.lastname: Lower
authors.affiliation: Christ Hospital, Cincinnati, OH.
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Engel
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D004311:Double-Blind Method; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D017565:Sarcoidosis, Pulmonary / Q000150:complications; D013449:Sulfonamides / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan