Table 1 Basic features of the included study (1)

Daniels CE 2010 [21]

Novartis Pharmaceuticals

59

60

78%

64%

66 (47–79)

67.8 (52–79)

——

——

64.4

65.6

Imatinib 600 mg /day

Placebo

Homma S 2012 [44]

None

38

38

76%

76%

67.6 ± 6.4

68.2 ± 7.7

3.0 ± 3.4

3.2 ± 2.5

89.2 ± 17.8

88.7 ± 15.5

N-acetylcysteine 704.8 mg /day

No treatment (or Placebo)

Jackson RM 2010 [20]

Pfizer UK

14

15

79%

80%

70 ± 12.1

71 ± 6.2

——

——

62.2 ± 16.7

62.7 ± 10.3

Sildenafil 60 mg /day

Placebo

King TE Jr(ASCEND)2014 [12]

Intermune

278

277

79.90%

76.90%

68.4±6.7

67.8±7.3

1.7±1.1

1.7±1.1

67.8±11.2

68.6±10.9

Pirfenidone 2403 mg/day

Placebo

King TE Jr(BUILD-1)2008 [39]

Actelion Pharmaceuticals

71

83

69%

75.9%

65.3±8.4

65.1±9.1

1.2±1.2

1.1±1.0

65.9 ± 10.5

69.5 ± 12.6

Bosentan 250 mg/day

Placebo

King TE Jr(BUILD-3)2011 [40]

Actelion Pharmaceuticals

407

209

72.70%

63.60%

63.8 ± 8.4

63.2 ± 9.1

0.48 (0.05–4.72)

0.50 (0.05–4.72)

74.9 ± 14.8

73.1 ± 15.3

Bosentan 250 mg/day

Placebo

Lancaster L 2020 [35]

None

56

57

80.4%

64.9%

68.8 ±7.6

66.2 ±9.4

1.5 ±1.4

1.5 ±1.4

78.0±17.4

78.1 ± 19.4

Nintedanib 300 mg /day

Placebo

Maher TM(FLORA)2018 [23]

Galapagos

17

6

59%

83%

67·0 (61·0–73·0)

64·0 (54·0–69·0)

1·9 (0·7–3·1)

1·0 (0·5–1·6)

75·3 (67·9–82·7)

69·7 (46·4–92·9)

GLPG1690 600 mg /day

Placebo

Maher TM(INMARK)2019 [36]

Boehringer Ingelheim

116

230

80%

73%

70·5 ±7·7

70·2 ±7·2

0·8 ±0·8

0·9 ±1·0

96·6±15·2

98·0 ± 12·6

Nintedanib 300 mg /day

Placebo

Martinez FJ 2014 [24]

Zambon SpA

133

131

80.50%

74.80%

68.3 ±8.4

67.2 ±8.2

1.0 ±1.0

1.1 ±1.0

72.2 ± 15.9

73.4 ± 14.3

N-acetylcysteine 1800mg /day

Placebo

Noble PW(CAPACITY 004)2011 [37]

Intermune

174

174

68%

74%

65·7 ±8·2

66·3 ±7·5

≤1 Y 48%

≤1 Y: 47%

74·5±14·5

76·2 ± 15·5

Pirfenidone 2403 mg/day

Placebo

Noble PW(CAPACITY 006)2011 [37]

Intermune

171

173

72%

72%

66·8 ±7·9

67·0 ±7·8

≤1 Y: 58%

≤1 Y: 62%

74·9 ±13·2

73·1 ± 14·2

Pirfenidone 2403 mg/day

Placebo

Noth I 2012 [25]

None

72

73

67%

79%

67.3 ± 7.1

66.7 ± 7.4

1.8 ± 1.9

2.1 ± 2.4

58.9 ± 16.2

58.7 ± 16.1

Warfarin (1 mg and 2.5 mg)/day

Placebo

Raghu G 2018 [22]

None

77

39

84%

74%

69.0 ±6.3

67.6 ±7.1

3.7 ±2.2

3.9 ±2.6

67.7±10.9)

67.4 ± 11.4

PRM-151 10 mg/kg/4 weeks

Placebo

Raghu G(ARTEMIS-IPF)2013 [26]

Gilead Sciences

329

163

74.20%

68.10%

65.8 ±7.4

66.1 ±7.1

1.1 ±1.4

0.9 ±1.2

68.7 ± 13.1

69.9 ± 13.8

Ambrisentan 10 mg/day

Placebo

Raghu G(MUSIC)2013 [41]

Actelion Pharmaceuticals

119

59

70.60%

62.70%

65.1±7.85

64.5±6.32

213 (3–1870)D

114 (2–1440)D

76.5 ± 15.6

74.8 ± 14.6

Macitentan 10 mg /day

Placebo

Raghu G(RAINIER)2017 [42]

Gilead Sciences Inc

272

272

84%

83%

67·7 ±7·6

68·5 ±7·1

2·0 ±2·1

2·0 ±2·3

61·4± 12·2

62·3 ± 12·2

Simtuzumab 125 mg/7 days

Placebo

Richeldi L 2020 [18]

FibroGen

50

53

66%

81%

68·3 ±7·1

68·4 ±7·2

1·1 ±1·0

1·5 ±1·2

74·5 ± 11·9

73·1 ± 11·1

Pamrevlumab 30 mg/kg/3 weeks

Placebo

Richeldi L(INPULSIS-1) 2014 [13]

Boehringer Ingelheim

309

204

81.20%

79.90%

66.9±8.4

66.9±8.2

1.7±1.4

1.6±1.4

79.5±17.0

80.5±17.3

Nintedanib 300 mg /day

Placebo

Richeldi L(INPULSIS-2) 2014 [13]

Boehringer Ingelheim

329

219

77.80%

78.10%

66.4±7.9

67.1±7.5

1.6±1.3

1.6±1.3

80.0±18.1

78.1±19.0

Nintedanib 300 mg /day

Placebo

Richeldi L(TOMORROW)2011 [14]

Boehringer Ingelheim

85

85

76.50%

74.10%

65.4±7.8

64.8±8.6

1.0±1.2

1.4±1.5

78.1

77.6

Nintedanib 300 mg /day

Placebo

Taniguchi 2010 [38]

Shionogi & Co., Ltd

108

104

78.70%

77.90%

65.4±6.2

64.7±7.3

≧1 Y: 64.9%

≧1 Y: 60.5%

77.3±16.8

79.1±17.4

Pirfenidone 1800 mg/day

Placebo

van den Blink B 2016 [43]

None

14

6

87%

67%

66.7±7.8

65.5±12.9

——

——

78.8±12.5

63.2±16.7

PRM-151 1, 5 or 10 mg/kg/days 1, 3, 5, 8 and 15

Placebo

Zisman DA(STEP-IPF)2010 [19]

Pfizer

89

91

84%

82%

69.76±8.71

68.20±9.25

2.03±1.94

1.87±1.93

54.89±14.00

58.73±14.12

Sildenafil 60 mg /day

Placebo