Summary of seven treatment trials in idiopathic pulmonary fibrosis(IPF)
| Trial | Study medication | Study design | Patients n | Inclusion criteria | Primary end-point | Ref. |
| BUILD-1 | Bosentan (125 mg b.i.d) versus placebo | 12-month, double-blind, randomised, placebo-controlled | 158 | IPF <3yrs; 6MWD limited by dyspnoea; moderate restrictive lung disease | Change from baseline in 6MWD | [6] |
| IFIGENIA | NAC (600 mg t.i.d) plus azathioprine plus prednisone versus azathioprine plus prednisone | 12-month, double-blind, randomised placebo-controlled | 182 | Change from baseline in FVC and DL,CO | [7] | |
| GIPF-001 | IFN-γ-1b (200 µg) versus placebo | Multicenter, double-blind, placebo-controlled | 330 | 20–79 yrs; IPF >3 yrs | Composite: progression-free survival | [8] |
| INSPIRE | IFN-γ-1b (200 µg) versus placebo | 2-yr, multicenter, double-blind, placebo-controlled | 800 | 4–79 yrs; IPF ≥3 months; mild-to-moderate IPF | Survival time from randomisation to treatment completion visit | [12] |
| Anticoagulant therapy | Warfarin or LMWH plus prednisolone versus prednisolone | Randomised, nonblinded | 56 | Biopsy-confirmed IPF and hospitalised for worsening IPF | Overall survival time to death and hospitalisation-free period | [9] |
| Pirfenidone | Pirfenidone (1800 mg·day−1) versus placebo | 12-month, double-blind, randomised, placebo-controlled | 107 | Confident clinical diagnosis IPF | Minimum Sp,O2during 6MWT | [10] |
| Etancerpt | Etanercept (25 mg sc) versus placebo | 12-month, double-blind, randomised, placebo-controlled | 87 | FVC <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.