|
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
|
