Independent factors associated with FVC decline ≥10% and all-cause death as determined by multivariable Cox regression
| Baseline characteristics | HR (95% CI) |
| FVC decline ≥10% | |
| Skin progression | 1.79 (1.20 to 2.65) |
| Age | 1.00 (0.99 to 1.01) |
| Male sex | 0.89 (0.67 to 1.19) |
| mRSS | 1.01 (0.99 to 1.03) |
| Disease duration | 1.00 (0.99 to 1.00) |
| Lung fibrosis on CT scan | 1.25 (0.90 to 1.72) |
| Pulmonary hypertension by echocardiography | 1.31 (0.93 to 1.85) |
| Dyspnoea NYHA stage ≥2 | 1.23 (0.94 to 1.62) |
| Joint synovitis | 1.10 (0.81 to 1.49) |
| FVC <70% predicted | 0.89 (0.64 to 1.24) |
| DLCO <70% predicted | 1.28 (0.97 to 1.69) |
| Anti-Scl-70 positive | 0.99 (0.75 to 1.29) |
| ACA positive | 1.07 (0.69 to 1.66) |
| CRP elevation | 1.22 (0.92 to 1.60) |
| All-cause death | |
| Skin progression | 2.58 (1.31 to 5.09) |
| Age | 1.05 (1.03 to 1.07) |
| Male sex | 1.56 (0.95 to 2.57) |
| Lung fibrosis on CT scan | 1.68 (0.84 to 3.36) |
| Pulmonary hypertension by echocardiography | 0.84 (0.47 to 1.50) |
| Renal crisis history | 3.15 (1.18 to 8.43) |
| Digital ulcers | 1.58 (0.99 to 2.53) |
| Proteinuria | 1.50 (0.74 to 3.04) |
| LVEF < 45% | 3.51 (1.22 to 10.12) |
| FVC <70% predicted | 2.60 (1.49 to 4.55) |
| DLCO <70% predicted | 2.00 (1.04 to 3.84) |
Factors highlighted in bold are significantly associated with the outcome.
Skin progression is defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months later.
ACA, anti-centromere antibody;Anti-Scl-70, anti-topoisomerase 1 antibody;CRP, C reactive protein;DLCO, diffusing capacity for carbon monoxide;FVC, forced vital capacity; LVEF, left ventricular ejection fraction;NYHA, New York Heart Association; mRSS, modified Rodnan skin score.