Table 1. Selected recent clinical trials in idiopathic pulmonary fibrosis
TrialDrug (putative MoA)Patients nEnd-pointOutcome
IFIGENIANAC (anti-oxidant; plus prednisone and AZT)155ΔVC, ΔDL,COPositive
NCT0063869Etanercept (TNF antagonist)88ΔFVC, ΔDL,CO, PA-a,O2Negative
INSPIREIFN-γ-1b (antifibrotic)826SurvivalNegative
BUILD-1Bosentan (endothelin inhibitor)1586MWTNegative
BUILD-3Bosentan (endothelin inhibitor)600Progression-free survivalNegative
NCT00131274Imatinib (tyrosin kinase inhibitor)119Disease progression or deathNegative
STEP-IPFSildenafil (PD5 inhibitor)1806MWTNegative
Shionogi phase IIIPirfenidone (antifibrotic)267ΔVCPositive
PIPF 004 (CAPACITY 2)Pirfenidone (antifibrotic)435ΔFVC % predPositive
PIPF 006 (CAPACITY 1)Pirfenidone (antifibrotic)344ΔFVC % predNegative
  • MoA: mechanism of action; NAC: N-acetylcysteine; AZT: azathioprine; TNF: tumour necrosis factor; IFN: interferon; PD5: phosphodiesterase-5; VC: vital capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; FVC: forced vital capacity; PA–a,O2: alveolar–arterial oxygen tension difference; 6MWT: 6-min walk test; % pred: % predicted.