Table 2.

Trials of pulmonary hypertension therapies in idiopathic pulmonary fibrosis.

Trial	Design	Medication/dose	Primary endpoint	Outcome
BUILD-1 (Bosentan Use in Interstitial Lung Disease)	Randomized, double-blind, placebo-controlled, multicenter study	Bosentan (oral) 62.5 mg b.i.d. × 4 wk., then 125 mg b.i.d. ≥ 12 mth.	6-minute-walk distance	Bosentan showed no superiority over placebo
STEP-IPF (Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis)	Randomized, double-blind, placebo-controlled trial	Sildenafil (oral) 20 mg t.i.d.	Proportion of patients with ≥20% increase in 6-minute-walk distance	Sildenafil showed no superiority over placebo in primary outcome
BUILD-3 (Bosentan Use in Interstitial Lung Disease)	Prospective, randomized, double-blind, placebo-controlled, event-driven, parallel-group trial	Bosentan (oral) 62.5 mg b.i.d. × 4 wk., then 125 mg b.i.d.,	Time to IPF worsening or death	No significant difference between treatment groups
ARTEMIS-IPF (Randomized, Placebo-Controlled Study to Evaluate Safety and Effectiveness of Ambrisentan in IPF)	Randomized, double-blind, placebo-controlled, event-driven phase 3 trial	Ambrisentan (oral) 10 mg daily	Reduction in rate of IPF progression	Early study termination due to worsening of lung function decline and increased respiratory hospitalizations in ambrisentan group
MUSIC (Macitentan Use in an Idiopathic Pulmonary Fibrosis Clinical Study)	Prospective, randomized, double-blind, multicenter, placebo-controlled, parallel-group phase 2 trial	Macitentan (oral) 10 mg daily	Effect on forced vital capacity	No differences in pulmonary function tests or time to disease progression or death
BPHIT (Bosentan in Pulmonary Hypertension Associated with Fibrotic Idiopathic Interstitial Pneumonia)	Randomized, double-blind, placebo-controlled phase 4 study	Bosentan (oral) 62.5 mg b.i.d. × 4 wk., then 125 mg b.i.d.	≥20% decrease from baseline of pulmonary vascular resistance index over 16 weeks	No difference in primary outcome