Patient characteristics
|
Age, years
|
54.0
|
14.4
|
67.6
|
8.8
|
<0.001
|
|
Height, cm
|
167.3
|
7.8
|
162.1
|
8.1
|
0.02
|
|
Weight, kg
|
70.0
|
16.0
|
66.0
|
10.5
|
n.s.
|
|
Body surface area, m2
|
1.77
|
0.2
|
1.7
|
0.2
|
n.s.
|
|
Duration of SSc, years
|
10.7
|
9.9
|
15.6
|
14.4
|
n.s.
|
|
Beginning of SSc, years
|
44.5
|
15.0
|
52.4
|
16.0
|
0.04
|
|
Modified Rodnan Skin Score
|
13.3
|
8.5
|
18.4
|
11.4
|
0.04
|
|
6MWD, m
|
466
|
77
|
349
|
86
|
<0.001
|
|
Borg Dyspnoea 20
|
13
|
2
|
15
|
2
|
0.003
|
WHO FC
|
0-I
|
17 (31.5 %)
| |
0
| | |
|
II
|
26 (48.1 %)
| |
5 (22.7 %)
|
III
|
10 (18.5 %)
| |
17 (77.3 %)
|
Unknown
|
1 (1.9 %)
| |
0
|
Laboratory
|
NT-proBNP, pg/mL
|
290
|
424
|
846
|
773.0
|
0.02
|
Blood gas analysis
|
Oxygen saturation, %
|
96.8
|
2.1
|
94.6
|
3.4
|
0.01
|
Lung function
|
Vital capacity, %
|
95.8
|
24.2
|
88.2
|
29.9
|
n.s.
|
|
FEV1, %
|
108.0
|
93.7
|
83.7
|
26.9
|
n.s.
|
|
Total lung capacity, %
|
95.5
|
21.4
|
83.8
|
23.2
|
0.042
|
|
Residual volume, %
|
2.0
|
0.8
|
1.9
|
0.6
|
n.s.
|
Diffusion capacity
|
Diffusion capacity carbon monoxide, %
|
55.7
|
13.5
|
42.1
|
12.5
|
<0.001
|
Echocardio-
|
RV thickness, mm
|
6.5
|
1.2
|
7.6
|
1.4
|
0.01
|
graphy
|
TAPSE, mm
|
24.0
|
3.5
|
20.6
|
4.0
|
0.001
|
|
RA area, cm2
|
11.4
|
3.3
|
15.5
|
5.7
|
<0.001
|
|
RV area, cm2
|
14.6
|
3.7
|
17.7
|
4.2
|
0.003
|
|
TRV, m/s
|
2.3
|
0.4
|
3.4
|
0.6
|
<0.001
|
|
PASP, mm Hg
|
25.6
|
7.3
|
52.0
|
18.0
|
<0.001
|
|
PASP max (exercise), mm Hg
|
49.9
|
12.7
|
83.9
|
18.9
|
<0.001
|
Right heart
|
RAP/CVP, mm Hg
|
3.8
|
2.6
|
6.3
|
4.5
|
0.02
|
catheterisation
|
mPAP, mm Hg
|
14.8
|
3.4
|
32.6
|
7.5
|
<0.001
|
at rest
|
PAWP, mm Hg
|
7.2
|
3.2
|
12.4
|
6.0
|
0.001
|
|
Cardiac output, ml/min
|
5.6
|
1.3
|
5.1
|
1.0
|
n.s.
|
|
Cardiac index, l/min per m2
|
3.1
|
0.6
|
2.9
|
0.5
|
n.s.
|
|
PVR, dynes*s/cm5
|
114
|
40
|
339
|
199
|
<0.001
|
During exercise
|
mPAP max, mm Hg
|
31
|
7
|
50
|
7
|
<0.001
|
|
PAWP max, mm Hg
|
17
|
6
|
21
|
9
|
n.s.
|
|
Cardiac output max, l/min
|
11.5
|
3.4
|
8.3
|
2.9
|
<0.001
|
|
PVR max, dynes*s/cm5
|
96
|
42
|
323
|
207
|
<0.001
|