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] |