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Table 2 Prognosis factors: non-adjusted and adjusted analysis on age at diagnosis, sex, and SSc subtype in the French cohort

From: Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature

  Non-adjusted HR p Adjusted HR p
Demographics
 Male sex 2.00 (1.31–3.05) 0.001 1.53 (0.98–2.39) 0.060
 Age at diagnosis (per 1 year) 1.05 (1.04–1.07) < 0.001 1.08 (1.04–1.12) < 0.001
 Age at diagnostic > 60 years 4.97 (2.53–9.78) < 0.001 5.79 (2.92–11.49) < 0.001
 Disease duration at time of diagnosis (per 1 year) 1.02 (0.97–1.07) 0.542 1.01 (0.96–1.06) 0.763
 African origin (vs. European) 0.79 (0.38–1.62) 0.516 0.93 (0.43–2.03) 0.864
Skin involvement
 dcSSc subtype (vs. lcSSc) 2.06 (1.39–3.05) < 0.001 2.40 (1.58–3.64) < 0.001
 mRSS > 5 1.24 (1.12–1.38) < 0.001 1.21 (1.03–1.43) 0.022
 Past and/or active digital ulcers 1.22 (0.79–1.90) 0.371 1.29 (0.81–2.04) 0.277
 Telangiectasia 1.64 (1.08–2.48) 0.019 1.55 (1.02–2.35) 0.039
 Calcinosis 1.37 (0.79–2.36) 0.260 1.22 (0.69–2.16) 0.503
Lung involvement
 NYHA class I –   –  
 NYHA class II 2.68 (1.46–4.92) 0.001 2.37 (1.29–4.36) 0.006
 NYHA class III 17.53 (3.97–14.27) < 0.001 6.74 (3.53–12.88) < 0.001
 NYHA class IV 25.76 (10.55–62.92) < 0.001 16.61 (6.68–41.26) < 0.001
 NYHA classes III–IV (vs. class I) 4.68 (3.07–7.13) < 0.001 4.33 (2.82–6.66) < 0.001
 6MWD (per 100 m) 0.46 (0.36–0.58) < 0.001 0.51 (0.39–0.67) < 0.001
 TLC < 70% predicted 3.87 (2.36–6.35) < 0.001 3.38 (1.96–5.82) < 0.001
 FVC < 70% predicted 3.11 (1.92–5.02) < 0.001 2.79 (1.62–4.80) < 0.001
 DLCO < 70% predicted 4.01 (2.33–6.89) < 0.001 3.31 (1.87–5.88) < 0.001
 Interstitial lung disease 1.99 (1.32–2.99) < 0.001 1.50 (0.96–2.34) 0.072
PH (echo. and/or RHC) 5.01 (3.18–7.89) < 0.001 4.15 (2.59–6.65) < 0.001
 sPAP < 35 mmHg –   –  
 35–46 mmHg 2.05 (0.98–4.28) 0.056 1.26 (0.58–2.70) 0.559
 > 46 mmHg 6.44 (3.69–11.22) < 0.001 5.94 (3.30–10.72) < 0.001
 PAH (RHC) 4.96 (2.82–8.72) < 0.001 4.39 (2.43–7.93) < 0.001
Heart involvement
 Arrhythmia 2.44 (0.98–6.02) 0.054 1.31 (0.52–3.32) 0.569
 AV block 0.95 (0.13–6.80) 0.956 1.15 (0.15–8.58) 0.890
 BB block 1.26 (0.31–5.15) 0.748 1.37 (0.33–5.67) 0.661
 LVEF < 50% 1.82 (0.25–13.24) 0.555 0.92 (0.12–6.84) 0.938
 Diastolic dysfunction 1.36 (0.43–4.35) 0.603 0.97 (0.30–3.13) 0.953
 Pericarditis 1.74 (0.84–3.61) 0.139 1.07 (0.50–2.26) 0.864
 Valvular disease 4.03 (1.97–8.25) < 0.001 2.20 (1.05–4.60) 0.037
Renal involvement
 Scleroderma renal crisis 3.44 (2.01–5.89) < 0.001 2.95 (1.61–5.40) < 0.001
 GFR < 80 ml/min 1.64 (1.06–2.52) 0.025 1.37 (0.85–2.21) 0.199
Gastrointestinal involvement 1.07 (0.68–1.69) 0.756 1.02 (0.65–1.62) 0.916
 BMI < 18.5 kg/m2 1.10 (0.45–2.74) 0.831 1.79 (0.71–4.51) 0.220
 Albuminemia < 35 g/l 2.30 (1.24–4.30) 0.009 1.45 (0.75–2.82) 0.270
Muscular involvement 1.66 (1.10–2.51) 0.016 1.46 (0.92–2.31) 0.106
 CPK > 200 IU/L 1.27 (0.58–2.76) 0.550 1.15 (0.50–2.64) 0.740
Joint involvement 1.22 (0.82–1.80) 0.329 1.08 (0.70–1.66) 0.720
Cancer 2.44 (1.41–4.21) 0.001 1.86 (1.07–3.26) 0.029
Anemia 2.66 (1.75–4.06) < 0.001 2.37 (1.54–3.66) < 0.001
CRP > 8 mg/l 2.05 (1.28–3.27) 0.003 1.70 (1.02–2.82) 0.041
Serologic features
 ACA 0.95 (0.62–1.44) 0.795 0.85 (0.55–1.31) 0.459
 Anti-Scl70 antibodies 0.87 (0.55–1.36) 0.534 0.82 (0.51–1.30) 0.390
 Anti-U1RNP antibodies 1.41 (0.51–3.93) 0.506 1.32 (0.44–3.92) 0.616
 Anti-RNAP3 antibodies 0.96 (0.23–3.94) 0.949 1.32 (0.44–3.92) 0.616
 Anti-PMScl antibodies 0.33 (0.05–2.41) 0.277 0.49 (0.07–3.54) 0.476
 APL antibodies 1.54 (0.71–3.35) 0.280 1.18 (0.53–2.63) 0.679
 Low complement 2.40 (0.97–5.95) 0.059 2.38 (0.95–5.95) 0.063
Smoking 1.06 (0.69–1.62) 0.795 0.97 (0.59–1.59) 0.901
  1. Results are expressed as hazard ratios and 95% confidence interval
  2. lcSSc limited cutaneous systemic sclerosis, dcSSc diffuse cutaneous systemic sclerosis, mRSS modified Rodnan score, GFR glomerular filtration rate, AV block atrioventricular block, BB block bundle branch block, LVEF left ventricular ejection fraction, PH pulmonary hypertension, PAH pulmonary arterial hypertension, echo echocardiography, RHC right heart catheterization, 6MWD 6-min walking distance, sPAP systolic pulmonary arterial pressure, HRCT high-resolution computer tomography, TLC total lung capacity, FVC forced vital capacity, DLCO diffusing capacity of the lungs for carbon monoxide, CRP C reactive protein, BMI body mass index, ACA anti-centromere antibodies, APL antiphospholipid antibodies
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