Female sex
|
5.42
|
1.13; 9.71
|
0.013
|
−3.36
|
−6.90; 0.18
|
0.063
|
13.56
|
6.41; 20.72
|
< 0.001
|
− 1.47
|
−2.60; − 0.33
|
0.011
|
Age
|
0.00
|
−0.19; 0.20
|
0.963
|
−0.18
|
− 0.34; − 0.02
|
0.029
|
0.35
|
0.04; 0.66
|
0.026
|
−0.05
|
− 0.10; 0.00
|
0.074
|
Age at first symptom onset in years
|
−0.10
|
− 0.28; 0.08
|
0.285
|
− 0.08
|
− 0.22; 0.07
|
0.323
|
0.17
|
− 0.11; 0.46
|
0.238
|
− 0.03
|
− 0.07; 0.02
|
0.299
|
Age at diagnosis in years
|
−0.09
|
− 0.28; 0.09
|
0.320
|
− 0.08
|
− 0.23; 0.07
|
0.290
|
0.18
|
− 0.11; 0.48
|
0.227
|
− 0.02
|
− 0.07; 0.03
|
0.400
|
Duration since first symptoms in years
|
0.73
|
0.28; 1.18
|
0.002
|
−0.51
|
−0.89; − 0.13
|
0.008
|
0.71
|
− 0.04; 1.46
|
0.062
|
− 0.09
|
− 0.21; 0.03
|
0.139
|
Disease duration in months
|
1.36
|
0.70; 2.02
|
< 0.001
|
−1.10
|
− 1.65; −0.55
|
< 0.001
|
1.70
|
0.64; 2.77
|
0.002
|
−0.25
|
−0.43; − 0.08
|
0.005
|
< 3 months
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
3 to < 6 months
|
−2.63
|
−9.30; 4.04
|
0.440
|
1.30
|
−4.28; 6.89
|
0.647
|
−4.48
|
−16.06; 7.11
|
0.449
|
0.54
|
−1.27; 2.34
|
0.560
|
More than 6 months
|
6.54
|
2.53; 10.55
|
0.001
|
−5.10
|
−8.43; − 1.76
|
0.003
|
8.43
|
1.74; 15.12
|
0.014
|
−0.68
|
−1.76; 0.40
|
0.215
|
Number of comorbidities
|
None
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
1
|
3.55
|
−1.37; 8.47
|
0.157
|
−5.19
|
−9.23; − 1.16
|
0.012
|
9.93
|
2.05; 17.80
|
0.013
|
−0.86
|
−2.16; 0.44
|
0.195
|
2
|
4.49
|
−0.63; 9.61
|
0.086
|
−6.46
|
−10.66; −2.26
|
0.003
|
10.11
|
1.88; 18.34
|
0.016
|
−1.02
|
−2.37; 0.33
|
0.140
|
3
|
5.87
|
−0.04; 11.78
|
0.051
|
−7.75
|
− 12.62; −2.88
|
0.002
|
15.37
|
5.74; 24.99
|
0.002
|
−1.98
|
−3.55; −0.41
|
0.013
|
4+
|
17.18
|
10.14; 24.23
|
< 0.001
|
−14.99
|
−20.88; −9.11
|
< 0.001
|
33.06
|
20.77; 45.35
|
< 0.001
|
−4.35
|
−6.24; −2.46
|
< 0.001
|
NHYA
|
I
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
II
|
16.07
|
9.16; 22.98
|
< 0.001
|
−12.61
|
−19.06; −6.15
|
< 0.001
|
22.28
|
10.78; 33.77
|
< 0.001
|
−1.86
|
−3.92; 0.21
|
0.078
|
III
|
26.95
|
19.97; 33.93
|
< 0.001
|
−18.00
|
−24.49; − 11.50
|
< 0.001
|
38.29
|
26.65; 49.92
|
< 0.001
|
−3.82
|
−5.90; − 1.75
|
< 0.001
|
IV
|
33.28
|
16.44; 50.13
|
< 0.001
|
−27.35
|
−41.90; − 12.81
|
< 0.001
|
51.63
|
24.63; 78.64
|
< 0.001
|
−8.69
|
−13.43; − 3.95
|
< 0.001
|
6MWD (m)
|
− 0.01
|
− 0.01; 0.00
|
0.217
|
0.00
|
0.00; 0.01
|
0.313
|
−0.01
|
− 0.03; 0.00
|
0.109
|
0.00
|
0.00; 0.00
|
0.423
|
FEV1% predicted
|
−0.55
|
−0.62; − 0.49
|
< 0.001
|
0.43
|
0.37; 0.49
|
< 0.001
|
− 0.80
|
− 0.91; − 0.70
|
< 0.001
|
0.09
|
0.07; 0.11
|
< 0.001
|
FVC % predicted
|
− 0.60
|
− 0.66; − 0.53
|
< 0.001
|
0.46
|
0.39; 0.53
|
< 0.001
|
−0.89
|
−1.00; − 0.78
|
< 0.001
|
0.11
|
0.09; 0.13
|
< 0.001
|
DLCO % predicted
|
−0.26
|
− 0.31; − 0.20
|
< 0.001
|
0.26
|
0.20; 0.33
|
< 0.001
|
− 0.35
|
− 0.44; − 0.25
|
< 0.001
|
0.04
|
0.02; 0.06
|
< 0.001
|
Change in FVC % predicted between baseline and 1-year follow-up
|
Stable/increase
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
Decrease by 0 to 10%
|
3.71
|
−0.55; 7.97
|
0.087
|
−2.90
|
−6.58; 0.77
|
0.122
|
4.08
|
−3.13; 11.29
|
0.267
|
−0.51
|
−1.67; 0.65
|
0.393
|
Decrease by > 10%
|
9.08
|
2.48; 15.67
|
0.007
|
−8.07
|
−13.81; −2.33
|
0.006
|
11.24
|
0.32; 22.16
|
0.044
|
−1.30
|
−3.09; 0.50
|
0.156
|
Change in DLCO % predicted between baseline and 1-year follow-up
|
Stable/increase
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
Decrease by 0 to 10%
|
5.46
|
0.85; 10.08
|
0.020
|
−0.41
|
−4.43; 3.60
|
0.841
|
10.46
|
2.85; 18.07
|
0.007
|
−0.37
|
−1.62; 0.88
|
0.561
|
Decrease by > 10%
|
7.79
|
0.85; 14.73
|
0.028
|
−3.93
|
−9.96; 2.10
|
0.201
|
10.74
|
−0.73; 22.21
|
0.067
|
−0.40
|
−2.28; 1.47
|
0.673
|
Change in FVC % predicted between baseline and last follow-up
|
Stable/increase
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
Decrease by 0 to 10%
|
3.44
|
−1.04; 7.92
|
0.133
|
−3.30
|
−7.08; 0.48
|
0.087
|
3.18
|
−4.45; 10.81
|
0.414
|
−0.22
|
−1.44; 0.99
|
0.719
|
Decrease by > 10%
|
7.47
|
2.11; 12.83
|
0.006
|
−6.50
|
−11.02; − 1.99
|
0.005
|
8.80
|
−0.15; 17.75
|
0.054
|
−0.87
|
−2.32; 0.59
|
0.242
|
Change in DLCO % predicted between baseline and last follow-up
|
Stable/increase
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
Decrease by 0 to 10%
|
4.41
|
−0.52; 9.35
|
0.080
|
−1.42
|
−5.59; 2.75
|
0.505
|
11.09
|
2.94; 19.23
|
0.008
|
−0.44
|
−1.77; 0.89
|
0.518
|
Decrease by > 10%
|
5.10
|
−0.51; 10.72
|
0.075
|
−2.47
|
−7.18; 2.25
|
0.305
|
8.24
|
−0.88; 17.36
|
0.077
|
−0.30
|
−1.80; 1.21
|
0.701
|
GAP index
|
3.29
|
1.95; 4.64
|
< 0.001
|
−3.41
|
−4.50; −2.33
|
< 0.001
|
6.47
|
4.36; 8.58
|
< 0.001
|
−0.50
|
− 0.86; − 0.14
|
0.007
|
Stage I
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
Stage II
|
5.69
|
0.74; 10.63
|
0.024
|
−7.04
|
−11.00; −3.08
|
< 0.001
|
16.51
|
8.69; 24.34
|
< 0.001
|
−0.92
|
−2.25; 0.41
|
0.177
|
Stage III
|
12.93
|
7.42; 18.45
|
< 0.001
|
−13.51
|
− 17.96; −9.07
|
< 0.001
|
25.78
|
17.08; 34.47
|
< 0.001
|
− 1.79
|
−3.27; −0.30
|
0.019
|
Overall physician’s judgement of clinical course of IPF
|
Stable disease
|
1.00
| | |
1.00
| | |
1.00
| | |
1.00
| | |
Slow progression
|
7.79
|
3.51; 12.06
|
< 0.001
|
−3.52
|
−7.14; 0.10
|
0.056
|
11.13
|
4.04; 18.21
|
0.002
|
−1.08
|
−2.23; 0.08
|
0.068
|
Rapid progression
|
13.16
|
6.22; 20.11
|
< 0.001
|
−9.52
|
−15.39; −3.65
|
0.001
|
19.89
|
8.32; 31.47
|
0.001
|
−2.72
|
−4.61; −0.83
|
0.005
|
No judgement possible
|
−0.39
|
−4.93; 4.15
|
0.865
|
1.33
|
−2.43; 5.09
|
0.487
|
1.72
|
−5.72; 9.15
|
0.651
|
−0.01
|
−1.23; 1.20
|
0.986
|