Predictors of low survival in the multiple variable model
| Mode | Simplified score | ||||
| Full | Final | ||||
| HR (95% CI) | p | HR (95% CI) p | p | ||
| Age, year | |||||
| 50–65 | 1.93 (1.6 to 2.32) | <0.001 | 1.86 (1.56 to 2.21) | <0.001 | 3 |
| >65 | 3.91 (3.2 to 4.78) | <0.001 | 3.63 (3.02 to 4.38) | <0.001 | 6 |
| Male sex | 1.37 (1.15 to 1.64) | <0.001 | 1.34 (1.13 to 1.58) | 0.001 | 1 |
| Diffuse cutaneous disease | 0.79 (0.67 to 0.93) | 0.006 | 1.25 (1.08 to 1.46) | 0.004 | 1 |
| >5 years disease duration | 0.91 (0.79 to 1.06) | 0.23 | – | – | – |
| Progressive digital vasculopathy* | 0.91 (0.66 to 1.27) | 0.58 | – | – | – |
| Oesophageal or gastric disease manifestations | 1.04 (0.88 to 1.23) | 0.65 | – | – | – |
| Intestinal involvement | 1.13 (0.97 to 1.32) | 0.13 | – | – | – |
| Systemic hypertension | 0.94 (0.79 to 1.11) | 0.46 | – | – | – |
| Scleroderma renal crisis | 1.56 (1.05 to 2.32) | 0.029 | 1.48 (1.02 to 2.15) | 0.039 | 2 |
| Palpitations | 1.14 (0.97 to 1.35) | 0.12 | – | – | – |
| Prominent dyspnoea | 1.81 (1.41 to 2.31) | <0.001 | 1.79 (1.43 to 2.24) | <0.001 | 3 |
| Digital ulcers | 1.27 (1.1 to 1.47) | 0.001 | 1.24 (1.08 to 1.42) | 0.002 | 1 |
| Joint synovitis | 1.00 (0.83 to 1.21) | 0.98 | – | – | – |
| Contracture | 1.3 (1.1 to 1.52) | 0.002 | 1.28 (1.1 to 1.49) | 0.001 | 1 |
| Tendon friction rub | 0.96 (0.77 to 1.21) | 0.75 | – | – | – |
| Muscle weakness | 1.3 (1.1 to 1.54) | 0.002 | 1.34 (1.14 to 1.56) | <0.001 | 1 |
| Elevated C reactive protein | 2.47 (1.93 to 3.15) | <0.001 | 2.34 (1.88 to 2.93) | <0.001 | 4 |
| Elevated creatine kinase | 1.09 (0.86 to 1.38) | 0.49 | – | – | – |
| Proteinuria | 2.04 (1.59 to 2.61) | <0.001 | 1.95 (1.53 to –2.47) | <0.001 | 3 |
| Left ventricular ejection fraction <50% | 1.46 (1.07 to –2.01) | 0.019 | 1.41 (1.04 to 1.91) | 0.027 | 2 |
| Pulmonary arterial hypertension*† | 1.13 (0.65 to 1.95) | 0.67 | – | – | – |
| Interstitial lung disease | 1.28 (1.09 to 1.5) | 0.003 | 1.26 (1.08 to 1.46) | 0.003 | 1 |
| Carbon monoxide diffusion capacity <60% predicted | 2.07 (1.75 to 2.44) | <0.001 | 2.02 (1.72 to 2.38) | <0.001 | 4 |
| Forced vital capacity <70% predicted | 1.41 (1.13 to 1.76) | 0.003 | 1.4 (1.13 to 1.73) | 0.002 | 2 |
| Disease activity score =3 | 0.85 (0.63 to 1.14) | 0.28 | – | – | – |
| Antinuclear antibodies | 1.04 (0.76 to 1.45) | 0.79 | – | – | – |
| Anti-Scl70 antibodies | 0.98 (0.83 to 1.16) | 0.8 | – | – | – |
*In the last month, dyspnoea was classified as prominent in presence of New York Heart Association functional class III or IV.
†Diagnosed at time of right heart catheterisation; interstitial lung disease was considered present if visible on chest radiograph or on high-resolution CT scan; disease was active if the disease activity score was ≥3; the full model contains all variables included in the multiple variable model. The final model is model after variable selection. The HRs are pooled over the 50 imputed data sets and divided by the calibration slope of 0.94. Simplified score points were attributed to the variables of the final model by rounding the regression coefficients multiplied by 5.