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Table 4 Characteristics of patients with SSc by PAH status

From: Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme

  PAH No PAH P value
mean ± SD or % mean ± SD or %
Number of patients 209 1283  
Age at recruitment, years 63.1 ± 10.4 56.3 ± 12.7 <0.001
Disease duration at recruitment, yearsa 13.6 ± 11.5 10.4 ± 9.9 <0.001
Female 87.1% 86.2% 0.88
Limited disease subtype 73.9% 70.3% 0.43
Anti-centromere pattern ANA 52.6% 44.1% 0.02
Scl 70 positive 6.9% 15.2% 0.003
RNA polymerase III positive 14.4% 12.9% 0.65
Digital ulcers ever 53.0% 41.9% 0.003
Telangiectasia ever 91.4% 82.4% <0.001
Calcinosis ever 53.3% 34.7% <0.001
Joint contractures ever 45.8% 35.7% 0.007
GORD 45.9% 45.3% 0.48
Bowel dysmotility 27.3% 22.7% 0.15
Anal incontinence 32.1% 25.7% 0.05
WHO Functional Class
 Class I 4 (2.1%) 467(39.1%) <0.001
 Class II 25 (12.9%) 469 (39.2%)
 Class III 106 (54.9%) 238 (19.9%)
 Class IV 58(30.1%) 22(1.8%)
NT-pro-BNP 218.9 ± 285.7 75.1 ± 159.8 0.005
  1. PAH pulmonary arterial hypertension, ANA antinuclear antibodies, 6MWD six minute walk distance, mPAP mean pulmonary arterial pressure, mRAP mean right atrial pressure, PVR pulmonary vascular resistance, GORD gastro-oesphageal reflux disease, WHO World Health Organisation, NT-pro-BNP N-terminal pro b-type natriuretic peptide
  2. aDisease duration defined as from first non-Raynaud’s disease manifestation
  3. Disease manifestations defined as present if ever present from systemic sclerosis (SSc) diagnosis