From: The economic burden of systemic sclerosis related pulmonary arterial hypertension in Australia
Patient characteristics (n=1,128) | PAH | No PAH | p value |
---|---|---|---|
mean ± SD or n(%) | mean ± SD or n(%) | ||
Number of patients | 153 (13.6%) | 975 (86.4%) | |
Demographics | |||
Age on SSc onseta, years | 49.3 ± 13.9 | 45.7 ± 13.9 | 0.004 |
Disease duration at recruitment, years | 14.5 ± 11.9 | 10.6 ± 9.9 | <0.01 |
Female | 134 (87.6%) | 794 (81.4%) | 0.06 |
Limited disease subtype | 115 (75.2%) | 684 (73.4%) | 0.65 |
Caucasian Ethnicity | 142 (94.7%) | 839 (94.8%) | 0.95 |
Follow-up, years | 4.6 ± 2.6 | 4.9 ± 2.5 | 0.16 |
Alive at censorship | 68 (44.4%) | 707 (72.5%) | <0.01 |
Autoantibody profile | |||
Anti-centromere pattern ANA | 77 (50.3%) | 406 (41.6%) | 0.01 |
Scl 70 +ve | 14 (9.2%) | 132 (13.5%) | 0.02 |
RNA polymerase III +ve | 12 (7.9%) | 87 (8.9%) | 0.07 |
Clinical manifestationsb | |||
Digital ulcers ever | 87 (57.3%) | 440 (47.2%) | 0.02 |
Telangiectasia ever | 141 (92.2%) | 815 (83.6%) | 0.006 |
Calcinosis ever | 89 (58.2%) | 389 (39.9%) | <0.01 |
GIT involvement | 133 (86.9%) | 802 (82.3%) | 0.15 |
Renal Crisis | 2 (1.3%) | 31 (3.3%) | 0.18 |
Pericardial Effusion | 28 (41.3%) | 124 (12.3%) | <0.01 |
Mild ILD (FVC>70%) | 42 (27.5%) | 208 (21.3%) | 0.09 |
6MWD | 289.6 ±115.3 | 439.1 ± 118.2 | <0.01 |
WHO Functional Class | |||
Class I | 2 (1.3%) | 275 (30.6%) | <0.01 |
Class II | 19 (12.6%) | 372 (41.4%) | |
Class III | 83 (55.0%) | 231 (25.7%) | |
Class IV | 47 (31.1%) | 21 (2.3%) | |
Brain Natriuretic Peptide | 295 ± 140 | 84.9 ± 18.0 | <0.01 |
N-terminal pro b-type Natriuretic Peptide | 1799 ± 1542 | 375 ± 110 | 0.03 |
Co-morbidities | |||
Angina | 32 (26.2%) | 121 (12.0%) | <0.01 |
CVA | 7 (11.1%) | 146 (13.7%) | 0.56 |
Diabetes Mellitus | 14 (15.7%) | 139 (13.4%) | 0.53 |
COAD | 23 (16.5%) | 130 (13.1%) | 0.27 |
Current smoker | 15 (10.4%) | 138 (14.1%) | 0.19 |