Prognostic factor | Estimate | Ref. |
Factors associated with survival | ||
HLA DRw6 | RR 54.52 (p=0.01) | [63] |
HLA DRw52 | RR not reported (p=0.02) | [63] |
Elevated mean Pra | HR 20.7 (p=0.0001) | [40] |
Higher mixed venous oxygen saturation | HR 0.17, 95% CI 0.09–0.33 | [44] |
PVR | No difference in Kaplan–Meier curves in patients with a change in PVR <20%, 20–34% or | [40] |
≥35% (p=0.8) | [55] | |
HR 1.10, 95% CI 1.03–1.18 (p<0.01) | [51] | |
PVR index: HR 1.05, 95% CI 1.01–1.09 (p<0.01) | [56] | |
HR 1.20, 95% CI 1.02–1.41 (p=0.02) | ||
SVI | HR 0.94, 95% CI 0.89–0.99 (p=0.02) | [55] |
Pulmonary artery capacitance | HR 0.43, 95% CI 0.20–0.91 (p=0.03) | [55] |
Functional class | Lower functional class has better survival: | [44] |
HR 2.26, 95% CI 1.02–4.97 | [56] | |
NYHA classes III/IV: HR 4.54, 95% CI 1.37–8.33 (p=0.01) | [55] | |
WHO classes III/IV have worse survival than classes I/II: | ||
log rank test p=0.02 | ||
Sex | Males have worse survival: HR 2.02, 95% CI 0.65–6.20¶ | [64] |
Females have better survival: HR 2.20, 95% CI 1.36–3.55 | [44] | |
Age yrs | Age >50 yrs has worse survival: HR 2.34, 95% CI 0.54–10.2¶ | [64] |
Younger age has better survival: HR 0.66, 95% CI 0.34–1.29 | [44] | |
Estimated glomerular filtration rate# | HR 2.63, 95% CI 1.29–5.37 (p<0.01)+ | [55] |
Signs of right heart failure | HR 2.56, 95% CI 1.02–14.28 (p=0.04) | [56] |
Factors not associated with survival | ||
Anti-centromere antibody | HR 1.67, 95% CI 0.66–4.26 | [64] |
No effect on survival: data not reported | [44] | |
Anti-Scl-70 | HR 0.28, 95% CI 0.03–1.99 | [64] |
No effect on survival: data not reported | [44] |
HLA: human leukocyte antigen; Pra: right atrial pressure; PVR: pulmonary vascular resistance; SVI: stroke volume index; NYHA: New York Heart Association; WHO: World Health Organization. #: <60 mL·min−1·1.73 m−2; ¶: estimate was suggestive but not statistically significant; +: estimate from univariate analysis. Multivariate models including different haemodynamic parameters estimate HR 2.56–3.41.