Table 4

Lung function worsening and mortality, expressed as hazard ratio (HR), in relation to baseline findings in the validation set (univariate analysis).

Time to FVC 10% DeclineTime to DLCO 15% DeclineMortality
HR (95% CI)pHR (95% CI)PHR (95% CI)p
IL-61.34 (1.12–1.6)0.0011.14 (0.96–1.36)0.131.32 (1.08–1.62)0.007
Sex1.19 (0.74–1.96)0.461.59 (1.02–2.49)0.041.36 (0.77–2.41)0.3
Age1.01 (0.99–1.02)0.481.02 (1.00–1.04)0.021.03 (1.00–1.05)0.004
Smoking1.06 (0.77–1.46)0.661.32 (0.98–1.8)0.071.14 (0.77–1.68)0.5
dcSSc1.59 (1.03–2.43)0.041.70 (1.11–2.59)0.011.26 (0.74–2.13)0.4
PH2.43 (1.33–4.44)0.0042.1 (1.13–3.9)0.023.85 (2.11–7.03)< 0.0001
DTPA0.98 (0.97–0.99)0.0040.99 (0.97–1.00)0.030.98 (0.96–1.00)0.06
FEV1, %0.98 (0.97–0.99)0.030.99 (0.98–1.00)0.520.98 (0.97–0.99)0.003
FVC, %0.98 (0.97–0.99)0.020.99 (0.98–1.00)0.430.99 (0.97–0.99)0.005
TLC, %0.98 (0.96–0.99)0.020.99 (0.98–1.01)0.50.98 (0.96–1.00)0.01
DLCO, %0.98 (0.97–0.99)0.0070.99 (0.98–1.00)0.060.96 (0.94–0.97)< 0.0001
KCO, %0.98 (0.97–1.01)0.190.98 (0.97–1.00)0.10.94 (0.92–0.97)< 0.0001
ACA0.77 (0.4–1.5)0.441.17 (0.66–2.08)0.61.94 (1.06–3.53)0.03
ATA1.3 (0.86–2.01)0.21.03 (0.68–1.56)0.90.62 (0.36–1.06)0.08
ESR1.01 (0.99–1.02)0.21 (0.99–1.02)0.71.02 (1.01–1.03)0.001
CPI1.02 (1.00–1.03)0.0081.01 (1.0–1.03)0.121.04 (1.03–1.06)< 0.0001
  • dcSSc: diffuse cutaneous SSc; PH: presence of pulmonary hypertension; CPI: composite physiologic index 18; ACA: anticentromere antibodies; ATA: antitopoisomerase antibodies; FVC: forced vital capacity; FEV1: forced expiratory volume 1 second; IL: interleukin; DTPA:99mTc-technetium-labeled diethylene-triamine-pentacetate clearance; KCO: carbon monoxide transfer coefficient corrected for lung volume; ESR: erythrocyte sedimentation rate.