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Table 2 Demographic, clinical, and immunological profile

From: Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

 

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

  1. 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
  2. Obs.: # subgroup of all patients with SSc PAH and mPAP > 20 mmHg—< 25 mmHg, lately named bordeline (Bo-PAH)