Table. 1—

Summary of seven treatment trials in idiopathic pulmonary fibrosis(IPF)

TrialStudy medicationStudy designPatients nInclusion criteriaPrimary end-pointRef.
BUILD-1Bosentan (125 mg b.i.d) versus placebo12-month, double-blind, randomised, placebo-controlled158IPF <3yrs; 6MWD limited by dyspnoea; moderate restrictive lung diseaseChange from baseline in 6MWD[6]
IFIGENIANAC (600 mg t.i.d) plus azathioprine plus prednisone versus azathioprine plus prednisone12-month, double-blind, randomised placebo-controlled182Change from baseline in FVC and DL,CO[7]
GIPF-001IFN-γ-1b (200 µg) versus placeboMulticenter, double-blind, placebo-controlled33020–79 yrs; IPF >3 yrsComposite: progression-free survival[8]
INSPIREIFN-γ-1b (200 µg) versus placebo2-yr, multicenter, double-blind, placebo-controlled8004–79 yrs; IPF ≥3 months; mild-to-moderate IPFSurvival time from randomisation to treatment completion visit[12]
Anticoagulant therapyWarfarin or LMWH plus prednisolone versus prednisoloneRandomised, nonblinded56Biopsy-confirmed IPF and hospitalised for worsening IPFOverall survival time to death and hospitalisation-free period[9]
PirfenidonePirfenidone (1800 mg·day−1) versus placebo12-month, double-blind, randomised, placebo-controlled107Confident clinical diagnosis IPFMinimum Sp,O2during 6MWT[10]
EtancerptEtanercept (25 mg sc) versus placebo12-month, double-blind, randomised, placebo-controlled87FVC <45% pred; DL,CO >25% pred; Pa,O2>55 mmHg/Sp,O2 >88%FVC % pred; DL,CO % pred; A–a gradient[11]
  • BUILD-1: Bosentan Use in Interstitial Lung Disease; IFIGENIA: Idiopathic Pulmonary Fibrosis International Group Exploring N-acetylcysteine 1 Annual; GIPF-001: interferon gamma GIPF-001 trial; NAC: N-acetylcysteine; IFN: interferon; LMWH: low molecular weight heparin; 6MWD: 6-min walking distance; FVC: forced vital capacity; % pred: % predicted; DL,CO: diffusing capacity of the lung for carbon monoxide; Pa,O2: arterial oxygen tension; Sp,O2: arterial oxygen saturation measured by pulse oximetry; 6MWT: 6-min walk test; A–a gradient: alveolar–arterial oxygen gradient. 1 mmHg = 0.133 kPa.