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Table 1 Characteristics of SSc patients by PAH status

From: The economic burden of systemic sclerosis related pulmonary arterial hypertension in Australia

Patient characteristics
(n=1,128)
PAHNo PAHp value
mean ± SD or n(%)mean ± SD or n(%)
Number of patients153 (13.6%)975 (86.4%) 
Demographics
 Age on SSc onseta, years49.3 ± 13.945.7 ± 13.90.004
 Disease duration at recruitment, years14.5 ± 11.910.6 ± 9.9<0.01
 Female134 (87.6%)794 (81.4%)0.06
 Limited disease subtype115 (75.2%)684 (73.4%)0.65
 Caucasian Ethnicity142 (94.7%)839 (94.8%)0.95
 Follow-up, years4.6 ± 2.64.9 ± 2.50.16
 Alive at censorship68 (44.4%)707 (72.5%)<0.01
Autoantibody profile
 Anti-centromere pattern ANA77 (50.3%)406 (41.6%)0.01
 Scl 70 +ve14 (9.2%)132 (13.5%)0.02
 RNA polymerase III +ve12 (7.9%)87 (8.9%)0.07
Clinical manifestationsb
 Digital ulcers ever87 (57.3%)440 (47.2%)0.02
 Telangiectasia ever141 (92.2%)815 (83.6%)0.006
 Calcinosis ever89 (58.2%)389 (39.9%)<0.01
 GIT involvement133 (86.9%)802 (82.3%)0.15
 Renal Crisis2 (1.3%)31 (3.3%)0.18
 Pericardial Effusion28 (41.3%)124 (12.3%)<0.01
 Mild ILD (FVC>70%)42 (27.5%)208 (21.3%)0.09
 6MWD289.6 ±115.3439.1 ± 118.2<0.01
WHO Functional Class
 Class I2 (1.3%)275 (30.6%)<0.01
 Class II19 (12.6%)372 (41.4%)
 Class III83 (55.0%)231 (25.7%)
 Class IV47 (31.1%)21 (2.3%)
Brain Natriuretic Peptide295 ± 14084.9 ± 18.0<0.01
N-terminal pro b-type Natriuretic Peptide1799 ± 1542375 ± 1100.03
Co-morbidities
 Angina32 (26.2%)121 (12.0%)<0.01
 CVA7 (11.1%)146 (13.7%)0.56
 Diabetes Mellitus14 (15.7%)139 (13.4%)0.53
 COAD23 (16.5%)130 (13.1%)0.27
 Current smoker15 (10.4%)138 (14.1%)0.19
  1. Abbreviations: pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), gastrointestinal tract (GIT), six minute walk distance (6MWD), world health organization (WHO), health assessment questionnaire (HAQ), scleroderma HAQ (sHAQ), hypertension (HTN), cerebrovascular accident (CVA), peripheral vascular disease (PVD), chronic obstructive airways disease (COAD)
  2. adisease duration defined as from first non-Raynaud’s disease manifestation,
  3. bclinical manifestations defined as present if ever present from SSc diagnosis
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