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

 Male sex

2.00 (1.31–3.05)

1.53 (0.98–2.39)

 Age at diagnosis (per 1 year)

1.05 (1.04–1.07)

1.08 (1.04–1.12)

 Age at diagnostic > 60 years

4.97 (2.53–9.78)

5.79 (2.92–11.49)

 Disease duration at time of diagnosis (per 1 year)

1.02 (0.97–1.07)

1.01 (0.96–1.06)

 African origin (vs. European)

0.79 (0.38–1.62)

0.93 (0.43–2.03)

 dcSSc subtype (vs. lcSSc)

2.06 (1.39–3.05)

2.40 (1.58–3.64)

 mRSS > 5

1.24 (1.12–1.38)

1.21 (1.03–1.43)

 Past and/or active digital ulcers

1.22 (0.79–1.90)

1.29 (0.81–2.04)

 Telangiectasia

1.64 (1.08–2.48)

1.55 (1.02–2.35)

 Calcinosis

1.37 (0.79–2.36)

1.22 (0.69–2.16)

 NYHA class I

–

–

 NYHA class II

2.68 (1.46–4.92)

2.37 (1.29–4.36)

 NYHA class III

17.53 (3.97–14.27)

6.74 (3.53–12.88)

 NYHA class IV

25.76 (10.55–62.92)

16.61 (6.68–41.26)

 NYHA classes III–IV (vs. class I)

4.68 (3.07–7.13)

4.33 (2.82–6.66)

 6MWD (per 100 m)

0.46 (0.36–0.58)

0.51 (0.39–0.67)

 TLC < 70% predicted

3.87 (2.36–6.35)

3.38 (1.96–5.82)

 FVC < 70% predicted

3.11 (1.92–5.02)

2.79 (1.62–4.80)

 DLCO < 70% predicted

4.01 (2.33–6.89)

3.31 (1.87–5.88)

 Interstitial lung disease

1.99 (1.32–2.99)

1.50 (0.96–2.34)

PH (echo. and/or RHC)

5.01 (3.18–7.89)

4.15 (2.59–6.65)

 sPAP < 35 mmHg

–

–

 35–46 mmHg

2.05 (0.98–4.28)

1.26 (0.58–2.70)

 > 46 mmHg

6.44 (3.69–11.22)

5.94 (3.30–10.72)

 PAH (RHC)

4.96 (2.82–8.72)

4.39 (2.43–7.93)

 Arrhythmia

2.44 (0.98–6.02)

1.31 (0.52–3.32)

 AV block

0.95 (0.13–6.80)

1.15 (0.15–8.58)

 BB block

1.26 (0.31–5.15)

1.37 (0.33–5.67)

 LVEF < 50%

1.82 (0.25–13.24)

0.92 (0.12–6.84)

 Diastolic dysfunction

1.36 (0.43–4.35)

0.97 (0.30–3.13)

 Pericarditis

1.74 (0.84–3.61)

1.07 (0.50–2.26)

 Valvular disease

4.03 (1.97–8.25)

2.20 (1.05–4.60)

 Scleroderma renal crisis

3.44 (2.01–5.89)

2.95 (1.61–5.40)

 GFR < 80 ml/min

1.64 (1.06–2.52)

1.37 (0.85–2.21)

Gastrointestinal involvement

1.07 (0.68–1.69)

1.02 (0.65–1.62)

 BMI < 18.5 kg/m2

1.10 (0.45–2.74)

1.79 (0.71–4.51)

 Albuminemia < 35 g/l

2.30 (1.24–4.30)

1.45 (0.75–2.82)

Muscular involvement

1.66 (1.10–2.51)

1.46 (0.92–2.31)

 CPK > 200 IU/L

1.27 (0.58–2.76)

1.15 (0.50–2.64)

Joint involvement

1.22 (0.82–1.80)

1.08 (0.70–1.66)

Cancer

2.44 (1.41–4.21)

1.86 (1.07–3.26)

Anemia

2.66 (1.75–4.06)

2.37 (1.54–3.66)

CRP > 8 mg/l

2.05 (1.28–3.27)

1.70 (1.02–2.82)

 ACA

0.95 (0.62–1.44)

0.85 (0.55–1.31)

 Anti-Scl70 antibodies

0.87 (0.55–1.36)

0.82 (0.51–1.30)

 Anti-U1RNP antibodies

1.41 (0.51–3.93)

1.32 (0.44–3.92)

 Anti-RNAP3 antibodies

0.96 (0.23–3.94)

1.32 (0.44–3.92)

 Anti-PMScl antibodies

0.33 (0.05–2.41)

0.49 (0.07–3.54)

 APL antibodies

1.54 (0.71–3.35)

1.18 (0.53–2.63)

 Low complement

2.40 (0.97–5.95)

2.38 (0.95–5.95)

Smoking

1.06 (0.69–1.62)

0.97 (0.59–1.59)