| Prognostic factor | Estimate | Inference | Ref. |
| Ppa | Baseline systolic Ppa >60 mmHg: | Yes | [64] |
| HR 3.60, 95% CI 1.42–9.15 | Suggestive but not significant | [64] | |
| Increasing Ppa: HR 5.36, 95% CI 0.4–37.8 | Yes | [40] | |
| 3-yr survival in patients with P̄pa <32 mmHg, 32–44 mmHg and >45 mmHg 75, 61 and 33%, respectively | No | [65] | |
| (Kaplan–Meier estimate p<0.01) | |||
| P̄pa ≤35 mmHg versus >35 mmHg showed no difference | |||
| (Kaplan–Meier estimate log rank test p=0.08) | |||
| Limited sub-type | HR 2.37, 95% CI 0.68–8.20 | Suggestive but not significant | [64] |
| Limited and diffuse SSc have equally poor survival: 10–20% at 5 yrs | No | [67] | |
| (test of significance not reported) | |||
| Interstitial lung disease | No difference in Kaplan–Meier curves in SSc-PAH patients with and without fibrosis (p=0.3) | No | [40] |
| No difference in median survival in SSc-PAH with and without fibrosis: 55 months | No | [49] | |
| (95% CI 3–58 months) versus 11.5 months (95% CI 4–26 months); log rank p=0.20 | Yes | [44] | |
| 3-yr survival 47% versus 28%; p=0.005 | Yes | [51] | |
| HR 5.15, 95% CI 1.73–15.3 (p<0.01) 3-yr survival SSc-PH-ILD 47% versus SSc-PAH 71% (p=0.07) | Yes | [65] | |
| Time between SSc onset and observed PAH | 5.24 versus 9.93 yrs (p<0.01) | Yes | [68] |
| HR 0.99 95% CI 0.95–1.03 (p=0.60) | No | [55] | |
| Data not reported | No | [44] |
Ppa: pulmonary artery pressure; P̄pa: mean pulmonary artery pressure; PH: pulmonary hypertension; ILD: interstitial lung disease.