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| Trial | Design | Medication/dose | Primary endpoint | Outcome |
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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 |
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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 |
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