Table 1. Main characteristics of the randomized controlled trials included in the meta-analysis.
| Study, yr | Sample size | Age, yr | Men, % | Trial duration, mon | SGRQ total score | PAP, mmHg | FVC, % predicted | DLco, % predicted | 6MWD, m | Intervention | Concomitant IPF medications | Primary end-point |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Corte et al.24 | 60 | 66.6 | 70 | 4 | NR | 36.0 | 54.2 | 21.3 | 156.4 | Bosentan | Prednisone in 43 patients (71.7%), immunosuppressive agents in 25 (41.7%), and N-acetylcysteine in 43 (71.7%) | Decrease of 20% or more in pulmonary vascular resistance index compared to baseline |
| Han et al.15 | 119 | 67.7 | NR | 3 | NR | NR | 56.9 | 26.0 | 273.4 | Sildenafil | NR | Change in 6MWD from baseline |
| Jackson et al.31 | 29 | 70.5 | 79.3 | 6 | NR | NR | 62.5 | 42.0 | 346.8 | Sildenafil | Did not permit | Change in 6MWD or the Borg dyspnea index from baseline |
| King et al.14 | 154 | 65.2 | 72.7 | 12 | NR | NR | 67.8 | 41.8 | 373.4 | Bosentan | 15 mg or less of prednisone in 30 patients (19.5%) | Exercise capacity measured by a modified six minute-walk test |
| King et al.27 | 616 | 63.6 | 69.6 | 12 | NR | NR | 74.3 | 47.8 | NR | Bosentan | 20 mg or less of prednisone in 96 patients (15.6%) | IPF worsening (a confirmed decrease from baseline in FVC > 10% and DLco > 15% or acute exacerbation of IPF) or death |
| Kolb et al.25 | 274 | 70.2 | 79.1 | 6 | 55.3 | NR | 67.0 | 25.7 | NR | Sildenafil | Nintedanib | Change in SGRQ total score from baseline |
| Raghu et al.28 | 492 | 65.6 | 71.9 | 21 | 43.0 | 20.3 | 68.8 | 43.0 | 412.1 | Ambrisentan | Did not permit | Time to disease progression and a categorical decrease in lung function |
| Raghu et al.29 | 178 | 64.9 | 68.0 | 12 | NR | NR | 75.3 | 47.1 | NR | Macitentan | Prednisone in 43 patients (24.2%) and N-acetylcysteine in 32 (patients 18.0%) | Change in FVC from baseline to month 12 |
| Tanaka et al.26 | 24 | 68.7 | 70.8 | 24 | NR | 21.0 | 68.9 | 29.0 | 271.4 | Bosentan | Included drugs intended for the treatment of IPF except for pulmonary hypertension-specific agents (data not shown) | NR |
| Zisman et al.30 | 180 | 69.0 | 83.3 | 3 | 53.1 | NR | 56.8 | 26.3 | 258.1 | Sildenafil | Did not permit | Proportion of patients with a 20% or greater increase in 6MWD |
Data are presented as number or mean, unless otherwise stated.
6MWD = 6-minute walk distance test, DLco = diffusion capacity of the lungs for carbon monoxide, FVC = forced vital capacity, IPF = idiopathic pulmonary fibrosis, NR = not reported, PAP = pulmonary arterial pressure, SGRQ = St. George's Respiratory Questionnaire.