Overview of trials with targeted pulmonary hypertension (PH) medication in interstitial lung diseases mentioned in this article

TrialSubjects nStudy typeDrugsPatient populationDurationPrimary end-point resultOther outcomes
STEP-IPF (2010) [56]180RCTSildenafil versus placebo12 weeksNo improvement in 6MWTNo difference in death or exacerbation
Improvement in QoL
Dawes et al. (2021) [57]183RetrospectivePDE-5 versus no treatmentGroup 3 PH (67% ILD-PH)Median follow-up 1.6 yearsTransplant-free survival 2.33 versus 1.09 years
INSTAGE (2018) [58]247RCTSildefanil+nintedanib versus placebo+nintedanibIPF+PH24 weeksNo improvement in QoLReduction of death/decline in FVC
Behr et al. (2021) [59]247RCTSildenafil+pirfenidone versus placebo+pirfenidoneIPF+risk of PH52 weeksNo benefit for disease progression/death
Artemis-IPF (2013) [81]68RCTAmbrisentan versus placeboIPFTerminated earlyLack of efficacy and potential harm (more hospitalisation)
RISE-IIP (2019) [61]147RCTRiociguat versus placeboIIP+PHTerminated earlyIncreased SAE and death in patients with riociguat
INCREASE (2021) [62]326RCTTreprostinil inhalative versus placeboILD-PH16 weeksIncrease in 6MWTImprovement 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.