Table 5 Results of network meta-analysis of all-cause mortality

2.51 (0.52,12.20) ⊕⊕⊝⊝low2,4

Ambrisentan

2.79 (0.41,18.91) ⊕⊕⊝⊝low2,4

1.11 (0.21,5.95) ⊕⊕⊝⊝low2,4

N-acetylcysteine

4.73 (1.17,19.06)

⊕⊕⊝⊝low2,4

1.89 (0.66,5.36) ⊕⊕⊝⊝low2,4

1.69 (0.38,7.58) ⊕⊕⊕⊝moderate4

Bosentan

5.01 (0.84,29.68) ⊕⊕⊝⊝low2,4

2.00 (0.44,9.15) ⊕⊕⊝⊝low2,4

1.79 (0.28,11.55) ⊕⊕⊕⊝moderate4

1.06 (0.28,3.98) ⊕⊕⊕⊝moderate4

Macitentan

5.63 (1.54,20.55) ⊕⊕⊕⊝moderate2

2.25 (0.91,5.57) ⊕⊕⊕⊝moderate2

2.02 (0.49,8.24) ⊕⊕⊕⊕high

1.19 (0.71,1.99) ⊕⊕⊕⊕high

1.13 (0.33,3.82) ⊕⊕⊕⊕high

Placebo

5.84 (1.44,23.61) ⊕⊕⊝⊝low2,4

2.33 (0.82,6.65) ⊕⊕⊝⊝low2,4

2.09 (0.47,9.39) ⊕⊕⊝⊝low2,4

1.23 (0.59,2.58⊕⊕⊝⊝low2,4

1.17 (0.31,4.41) ⊕⊕⊝⊝low2,4

1.04 (0.61,1.75) ⊕⊕⊕⊝moderate2

Simtuzumab

7.18 (1.39,37.05) ⊕⊕⊝⊝low2,4

2.86 (0.74,11.12) ⊕⊕⊝⊝low2,4

2.57 (0.46,14.52) ⊕⊕⊕⊝moderate4

1.52 (0.49,4.71) ⊕⊕⊕⊝moderate4

1.43 (0.29,6.99) ⊕⊕⊕⊝moderate4

1.27 (0.47,3.49) ⊕⊕⊕⊕high

1.23 (0.39,3.83) ⊕⊕⊝⊝low2,4

Imatinib

8.17 (2.10,31.82) ⊕⊕⊝⊝low2,4

3.26 (1.20,8.85) ⊕⊕⊝⊝low2,4

2.92 (0.67,12.69) ⊕⊕⊕⊝moderate4

1.73 (0.89,3.35) ⊕⊕⊕⊝moderate4

1.63 (0.45,5.93) ⊕⊕⊕⊝moderate4

1.45 (0.96,2.20) ⊕⊕⊕⊕high

1.40 (0.72,2.74) ⊕⊕⊝⊝low2,4

1.14 (0.38,3.39) ⊕⊕⊕⊝moderate4

Pirfenidone

8.38 (2.14,32.84) ⊕⊕⊝⊝low2,4

3.34 (1.22,9.15) ⊕⊕⊝⊝low2,4

3.00 (0.69,13.09) ⊕⊕⊕⊝moderate4

1.77 (0.90,3.48) ⊕⊕⊕⊝moderate4

1.67 (0.46,6.12) ⊕⊕⊕⊝moderate4

1.49 (0.96,2.30) ⊕⊕⊕⊕high

1.44 (0.73,2.84) ⊕⊕⊝⊝low2,4

1.17 (0.39,3.50) ⊕⊕⊕⊝moderate4

1.03 (0.56,1.88) ⊕⊕⊕⊝moderate4

Nintedanib

11.26 (1.31,97.22) ⊕⊕⊝⊝low2,4

4.49 (0.64,31.50) ⊕⊕⊝⊝low2,4

4.03 (0.44,37.30) ⊕⊕⊕⊝moderate4

2.38 (0.39,14.39) ⊕⊕⊕⊝moderate4

2.25 (0.27,18.60) ⊕⊕⊕⊝moderate4

2.00 (0.36,11.20) ⊕⊕⊕⊕high

1.93 (0.32,11.69) ⊕⊕⊝⊝low2,4

1.57 (0.21,11.55) ⊕⊕⊕⊝moderate4

1.38 (0.23,8.12) ⊕⊕⊕⊝moderate4

1.34 (0.23,7.95) ⊕⊕⊕⊝moderate4

Sildenafil

11.26 (1.62,78.32) ⊕⊕⊝⊝low2,4

4.49 (0.82,24.72) ⊕⊕⊝⊝low2,4

4.03 (0.54,30.28) ⊕⊕⊕⊝moderate4

2.38 (0.51,11.03) ⊕⊕⊕⊝moderate4

2.25 (0.34,14.91) ⊕⊕⊕⊝moderate4

2.00 (0.47,8.47) ⊕⊕⊕⊕high

1.93 (0.42,8.97) ⊕⊕⊝⊝low2,4

1.57 (0.27,9.13) ⊕⊕⊕⊝moderate4

1.38 (0.31,6.20) ⊕⊕⊕⊝moderate4

1.34 (0.30,6.07) ⊕⊕⊕⊝moderate4

1.00 (0.11,9.47) ⊕⊕⊕⊝moderate4

Pamrevlumab