Overview of trials with targeted pulmonary hypertension (PH) medication in interstitial lung diseases mentioned in this article
| Trial | Subjects n | Study type | Drugs | Patient population | Duration | Primary end-point result | Other outcomes |
| STEP-IPF (2010) [56] | 180 | RCT | Sildenafil versus placebo | 12 weeks | No improvement in 6MWT | No difference in death or exacerbation Improvement in QoL | |
| Dawes et al. (2021) [57] | 183 | Retrospective | PDE-5 versus no treatment | Group 3 PH (67% ILD-PH) | Median follow-up 1.6 years | Transplant-free survival 2.33 versus 1.09 years | |
| INSTAGE (2018) [58] | 247 | RCT | Sildefanil+nintedanib versus placebo+nintedanib | IPF+PH | 24 weeks | No improvement in QoL | Reduction of death/decline in FVC |
| Behr et al. (2021) [59] | 247 | RCT | Sildenafil+pirfenidone versus placebo+pirfenidone | IPF+risk of PH | 52 weeks | No benefit for disease progression/death | |
| Artemis-IPF (2013) [81] | 68 | RCT | Ambrisentan versus placebo | IPF | Terminated early | Lack of efficacy and potential harm (more hospitalisation) | |
| RISE-IIP (2019) [61] | 147 | RCT | Riociguat versus placebo | IIP+PH | Terminated early | Increased SAE and death in patients with riociguat | |
| INCREASE (2021) [62] | 326 | RCT | Treprostinil inhalative versus placebo | ILD-PH | 16 weeks | Increase in 6MWT | Improvement NT-proBNP, time to clinical worsening |
RCT: randomised controlled trial; 6MWT: 6-min walk test; QoL: quality of life; PDE-5: phosphodiesterase-5; ILD: interstitial lung disease; FVC: forced vital capacity; IPF: idiopathic pulmonary fibrosis; IIP: idiopathic interstitial pneumonia; SAE: serious adverse event; NT-proBNP: N-terminal pro-brain natriuretic peptide.