Figures
- FIGURE 1
Study flow diagram. RCTs: randomised controlled trials.
- FIGURE 2
Pooled proportions of mortality at 1 year to <2 years. ES: effect size.
- FIGURE 3
Pooled proportions of mortality between 2–5 years. ES: effect size.
- FIGURE 4
Pooled proportions of mortality at ≥5 years. ES: effect size.
- FIGURE 5
Pooled mean changes in forced vital capacity (FVC) at 1 year to <2 years.
- FIGURE 6
Pooled mean changes in diffusing capacity of the lung for carbon monoxide (DLCO) at 1 year to <2 years.
Tables
- TABLE 1
Characteristics of studies identified
Characteristics Details Study type 154 cohort studies (64% retrospective design)#
16 RCTs
Publication year Before 1990: n=6
1990–1999: n=17
2000–2009: n=38
Since 2010: n=109
Location Europe: n=60
Asia: n=47
American continent: n=45
Australia: n=5
IPF diagnostic criteria 2011 ATS/ERS/JRS/ALAT guideline: n=41
2000 ATS/ERS international consensus statement: n=57
Other: n=72
Design Cohort study# Prospective design (n=55): 73% single-centre, 20% multicentre, 5% registry/database, 2% unclear
Retrospective design (n=98): 79% single-centre, 12% multicentre, 8% registry/database, 1% unclear
RCT (n=16): all multicentre trials Participant number Cohort study# Prospective design (n=55): median (range) 44 (7–588)
Retrospective design (n=98): median (range) 81 (11–13 615)
RCT (n=16): median (range) 94 (28–423)¶ Duration Cohort study#,+ Prospective design (n=55): 40% between 1 year and <2 years; 33% between 2–5 years; 13% ≥5 years
Retrospective design (n=98): 43% between 1 year and <2 years; 12% between 2–5 years; 10% ≥5 years
RCT (n=16): all with a study duration of 1 year and <2 years IPF: idiopathic pulmonary fibrosis; ATS: American Thoracic Society; ERS: European Respiratory Society; JRS: Japanese Respiratory Society; ALAT: Latin American Thoracic Association; RCT: randomised controlled trial. #: not including Mura et al. [110]: a single-centre study with both prospective (n=70) and retrospective (n=68) cohorts, with a study duration ≥3 years; ¶: number of participants in the placebo arm only; +:: all studies had a study duration ≥1 year. Some studies provided mean or median follow-up duration which could not be incorporated into the table.
- TABLE 2
Subgroup analyses for pooled analyses of the proportion of mortality according to study design
Studies Participants Proportion 95% CI Heterogeneity 1 year to <2 years Overall# 62 25 770 0.12 0.09–0.14 I2=95% RCT 16 2420 0.07 0.05–0.09 I2=75% Prospective study 18 769 0.11 0.05–0.18 I2=81% Retrospective study 28 22 581 0.16 0.13–0.19 I2=95% Test for heterogeneity between subgroups 0.36 2 to <5 years Overall 35 20 787 0.38 0.34–0.42 I2=94% Prospective study 18 1650 0.33 0.25–0.43 I2=92% Retrospective study 17 19 092 0.43 0.38–0.47 I2=93% Test for heterogeneity between subgroups=0.08 ≥5 years Overall 32 6369 0.69 0.59–0.78 I2=98% Prospective study 9 1060 0.58 0.38–0.78 I2=97% Retrospective study 23 5309 0.73 0.61–0.83 I2=98% Test for heterogeneity between subgroups=0.21 Data are presented as n, unless otherwise stated. RCT: randomised controlled trial. #: included data from RCTs.
- TABLE 3
Subgroup analyses for pooled analyses of the proportion of mortality
Studies Participants Proportion 95% CI Heterogeneity 1 year to <2 years Overall 62 25 770 0.12 0.09–0.14 I2=95% 2011 ATS/ERS/JRS/ALAT guideline 16 2283 0.07 0.05–0.09 I2=72% 2000 ATS/ERS consensus statement 25 2382 0.10 0.06–0.15 I2=92% Other criteria 21 21 105 0.18 0.15–0.22 I2=92% Test for difference between subgroups <0.0001 2 to <5 years Overall 35 20 787 0.38 0.34–0.42 I2=94% 2011 ATS/ERS/JRS/ALAT guideline 4 1026 0.25 0.08–0.47 I2=98% 2000 ATS/ERS consensus statement 13 995 0.42 0.37–0.47 I2=54% Other criteria 18 18 766 0.38 0.33–0.43 I2=94% Test for difference between subgroups=0.17 ≥5 years Overall 32 6369 0.69 0.59–0.78 I2=98% 2011 ATS/ERS/JRS/ALAT guideline 4 326 0.62 0.38–0.83 I2=93% 2000 ATS/ERS consensus statement 9 815 0.62 0.47–0.75 I2=94% Other criteria 19 5228 0.74 0.62–0.84 I2=98% Test for difference between subgroups=0.37 Data are presented as n, unless otherwise stated. ATS: American Thoracic Society; ERS: European Respiratory Society; JRS: Japanese Respiratory Society; ALAT: Latin American Thoracic Association.
Supplementary Material
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Supplementary_material ERR-0158-2019_Supplementary_material
