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Table 1 Characteristics of patients with and without SSc-PAH (n = 1579)

From: Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: results from a large multicenter cohort study

 

PAH

No PAH

p

n = 132

n = 1447

mean ± SD or %

mean ± SD or %

Female

84.9 %

112

86.7 %

1254

0.56

Age at recruitment, years

62.7 ± 10.3

56.6 ± 12.8

<0.001

Race

 Caucasian

 Asian

 Aboriginal-Islander

 Hispanic

 Other

112 (84.9 %)

6 (4.6 %)

1 (0.8 %)

1 (0.8 %)

0

1234 (85.3 %)

61 (4.2 %)

15 (1.1 %)

9 (0.6 %)

14 (0.9 %)

0.89

Follow-up duration

Total 3.2 (±2.5)

3.5 (±2.4)

3.1 (±2.5)

0.13

Age at PAH diagnosis

62.3 (±10.5)

n/a

 

Disease durationa at recruitment, years

Total: 11.1 (±10.2)

14.4 ± 12.1

10.8 ± 9.9

0.002

Disease subtype

 Limited

 Diffuse

 MCTD

91 (68.9 %)

30 (22.7 %)

7 (5.3 %)

959 (66.3 %)

353 (24.4 %)

70 (4.8 %)

0.82

Anti-centromere pattern ANA

63 (51.6 %)

584 (43.6 %)

0.10

Scl 70 positive

9 (7.4 %)

189 (14.1 %)

0.04

Antiphospholipid antibody

33 (27.1 %)

292 (21.8 %)

0.12

RNA polymerase III positive

8 (11.4 %)

100 (12.4 %)

0.57

SSc associated Disease manifestations

 GAVE

 Reflux oesphagitis

 Oesphageal stricture

 Oesphageal dysmotility

 Bowel dysmotility

 Anal incontinence

 Myocardial

 Pericardial effusion

 Renal crisis

 Myositis

 Synovitis

 Joint contractures

 Calcinosis

 Digital Ulcers

 Sicca symptoms

 ILD

 Telangiectasia

 Rodnan skin score (highest ever)

16 (12.2 %)

65 (49.2 %)

29 (21.9 %)

16 (12.1 %)

41 (31.1 %)

41 (31.1 %)

10 (7.6 %)

24 (18.2 %)

2 (1.5 %)

0

27 (20.5 %)

58 (43.9 %)

70 (53.0 %)

72 (54.6 %)

84 (63.6 %)

51 (38.6 %)

114 (86.4 %)

13.4 ± 9.6

128 (8.9 %)

688 (47.6 %)

142 (9.8 %)

127 (8.8 %)

338 (23.4 %)

340 (23.5 %)

97 (6.7 %)

72 (4.9 %)

37 (2.6 %)

29 (2.0 %)

298 (20.6 %)

478 (33.0 %)

473 (32.7 %)

592 (40.9 %)

730 (50.5 %)

353 (24.4 %)

1119 (77.3 %)

10.9 ± 9.5

0.21

0.71

<0.0001

0.20

0.05

<0.0001

0.51

<0.001

0.46

0.10

0.86

<0.001

<0.001

0.01

<0.001

<0.001

0.01

0.01

SF 36 score

 Physical functioning

 Role – physical

 Bodily pain

 General health

 Vitality

 Social functioning

 Role – emotional

 Mental health

35.2 ± 21.2

30.0 ± 34.7

66.2 ± 10.2

32.6 ± 16.9

43.5 ± 17.7

62.6 ± 25.0

55.3 ± 37.8

61.8 ± 21.7

57.9 ± 56.0

48.3 ± 39.9

46.3 ± 26.5

46.8 ± 21.4

49.3 ± 23.6

70.6 ± 24.9

67.8 ± 35.7

68.3 ± 19.4

<0.001

<0.001

<0.001

<0.001

0.03

0.01

0.01

0.01

  1. Abbreviations: GAVE gastric antral vascular ectasia, ILD interstitial lung disease, PAH pulmonary arterial hypertension
  2. adisease duration from first non-Raynaud manifestation
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