| |
Non-SScPH (n = 50)
|
SSc-ILD-PH (n = 2)
|
SSc-PH Group 2 (n = 2)
|
SSc-PAH (n = 11)
|
SSc-PAH# (Bo-PAH) (n = 5)
|
P value
|
Test
|
|---|
|
Age y–o (SD)
|
49.27 (14.4)
|
47 (9.8)
|
48.5 (12.0)
|
56.7 (9.7)
|
61.8 (9.4)
|
0.03
|
ANOVA (F = 2.99)
|
|
Female (%)
|
44 (88)
|
2 (100)
|
2 (100)
|
8 (73)
|
4 (80)
|
0.16
|
χ2 = 6.54
|
|
Afro-Brazilians (%)
|
15 (30)
|
2 (100)
|
2 (100)
|
6 (54)
|
3 (60)
|
< 0.0001
|
χ2 = 54.48
|
|
Duration in years (SD)
|
10.7
|
5 (0)
|
7.5 (3.5)
|
11.7
|
10.2
|
0.97
|
ANOVA (F = 0.07)
|
|
lcSSc (%)
|
20 (40)
|
0
|
0
|
5 (45)
|
2 (40)
|
0.82
|
χ2 = 54.48
|
|
dcSSc (%)
|
30 (60)
|
2 (100)
|
2 (100)
|
6 (54)
|
3 (60)
|
< 0.0001
|
χ2 = 28.62
|
|
Telangiectasia (%)
|
18 (36)
|
1 (50)
|
1 (50)
|
9 (81)
|
3 (60)
|
0.0005
|
χ2 = 20.05
|
|
Dyspnea (%)
|
9 (20)
|
2 (100)
|
1 (50)
|
9 (83)
|
4 (80)
|
< 0.0001
|
χ2 = 60.23
|
|
Urate mg/dL (SD)
|
4.37 (1.5)
|
4.3 (1.7)
|
4.25 (1.8)
|
5.58 (1.0)
|
4.78 (0.5)
|
0.03
|
ANOVA (F = 2.99)
|
|
Left axis deviation
|
1
|
0
|
0
|
0
|
1
|
0.78
|
χ2 = 0.50
|
|
FVC % (SD)
|
78.0 (17.0)
|
51.1 (2.8)
|
75.3 (7.0)
|
82.0 (13.2)
|
77.2 (12.4)
|
0.89
|
ANOVA (F = 0.20)
|
|
DLco% (SD)
|
71.6 (21.4)
|
25.6 (0.7)
|
40.2 (28)
|
43.7 (14.3)
|
43.2 (13.2)
|
< 0.0001
|
ANOVA (F = 12.91)
|
|
FVC/DLco (SD)
|
1.1 (0.6)
|
2.1 (0.9)
|
2.5 (2)
|
2.0 (0.6)
|
1.9 (0.4)
|
< 0.0001
|
ANOVA (F = 24.02)
|
|
Detected TRV (%)
|
33 (66)
|
2 (100)
|
2 (100)
|
11 (100)
|
5 (100)
|
0.04
|
χ2 = 9.92
|
|
mPAP on TTE (SD)
|
24.15 (5.8)
|
50.5 (50.9)
|
46.2 (22.3)
|
40.7 (12.3)
|
37.6 (10.2)
|
< 0.0001
|
ANOVA (F = 10.43)
|
|
Nucleolar ANA (%)
|
18 (36)
|
1 (50)
|
2 (100)
|
4 (36)
|
2 (40)
|
< 0.0001
|
χ2 = 56.55
|
|
Antibody positive (%)
|
18 (36)
|
1 (50)
|
0
|
6 (54)
|
2 (40)
|
0.19
|
χ2 = 4.71
|
|
Anti-Scl-70 positive (%)
|
9 (18)
|
1 (50)
|
0
|
2 (18)
|
1 (20)
|
< 0.0001
|
χ2 = 27.89
|
|
Anti-RNP (%)
|
2 (4)
|
0
|
0
|
1 (9)
|
0
|
0.16
|
χ2 = 1.93
|
|
Centromere (%)
|
8 (16)
|
0
|
0
|
3 (27)
|
1 (20)
|
0.23
|
χ2 = 2.95
|
|
Died (%)
|
4 (8)
|
2 (100)
|
2 (100)
|
4 (36)
|
3 (60)
|
< 0.0001
|
χ2 = 106.52
|
- lcSSc limited cutaneous systemic sclerosis, dcSSc diffuse cutaneous systemic sclerosis, Bo-PAH borderline pulmonary artery hypertension, FVC forced vital capacity, DLco diffusion capacity for carbon monoxide, TRV tricuspid regurgitation velocity, mPAP mean pulmonary arterial pressure, ANA antinuclear antibody, anti-Scl-70: anti-topoisomerase antibody; anti-RNP: anti-ribonucleoprotein antibody
- Obs.: # subgroup of all patients with SSc PAH and mPAP > 20 mmHg—< 25 mmHg, lately named bordeline (Bo-PAH)
