Treatment Lung disease Study/reference Comments Sildenafil Lung fibrosis including an IPF subgroup Ghofrani et al., 2002, [22 ] Improvement in hemodynamics and gas exchange Sildenafil IPF Collard et al., 2007, [23 ] Improvement in 6MWD in 57% of patients Sildenafil IPF Jackson et al., 2010, [24 ] No improvement in 6MWD Sildenafil IPF Madden et al., 2006, [25 ] Only 3 patients treated for 3 months and showed improvement in 6MWD and TTE parameters Sildenafil IPF The IPF Clinical Research Network, 2010, [26 ] There was no difference in 6MWD between the two groups, as a primary outcome measure Sildenafil COPD Rietema et al., 2008, [27 ] No improvement in stroke volume or exercise capacity Sildenafil Sarcoidosis Barnett et al., 2009, [28 ] In 9 patients treated with sildenafil out of 22 total, there was slight improvement in hemodynamics, 6MWD, and NYHA-FC Sildenafil Sarcoidosis Milman et al., 2008, [29 ] In 12 patients treated, who were listed for transplantation, there was a significant decrease in mPAP. No improvement found in 6MWD Sildenafil COPD Blanco et al., 2010, [30 ] In a RCT of 20 patients with COPD-associated PH, sildenafil improved acute pulmonary hemodynamics at rest and during exercise and deteriorated oxygenation Bosentan IPF BUILD-1 study, King et al., 2008, [31 ] Bosentan treatment in patients with IPF did not show superiority over placebo on 6MWD Bosentan IPF BUILD-3 study, King et al., 2011, [32 ] No treatment effects were observed on health-related quality of life or dyspnea. The primary objective was not met Bosentan COPD Stolz et al., 2008, [33 ] 30 patients with COPD were randomly assigned in a 2 : 1 ratio to receive either bosentan or placebo for 12 weeks. Bosentan did not improve 6MWD and deteriorated hypoxemia and functional class Bosentan COPD Valerio et al., 2009, [34 ] In a quite small sample size (
), there was benefit in PAP, PVR, and 6MWD. No improvement in GOLD IV patients Riociguat COPD Ghofrani et al., 2011, [35 ] In a quite small sample size (
), there was a trend of improvement in hemodynamics (abstract) IV epoprostenol Sarcoidosis Fisher et al., 2006, [36 ] In 5 patients treated with parenteral epoprostenol, there was improvement of NYHA-FC by one or two stages within 29 months IV prostacyclin COPD Archer et al., 1996, [37 ] Treatment of 7 mechanically ventilated patients for COPD exacerbation caused worsening of hypoxemia
