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Table 1 Demographics and clinical characteristics of 625 patients with SSc at baseline

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

 

N (N for dcSSc)

Total

dcSSc

lcSSc

p

Demographics

 Female sex

625/179

493 (79)

124 (69)

369 (83)

< 0.001

 Age at first RP (years)

554/155

45.4 ± 15.7

45.8 ± 15.7

45.3 ± 15.8

0.736

 Age at first non-RP symptom (years)

502/160

50.6 ± 14.5

48.5 ± 14.4

51.5 ± 14.4

0.031

 Age at diagnosis (years)

625/179

52.7 ± 14.9

49.5 ± 14.5

53.9 ± 14.9

< 0.001

 Disease duration from first non-RP symptom to diagnosis (years)

499/160

0.8 [2.2]

0.7 [1.4]

0.9 [2.7]

0.042

 Follow-up time from inclusion to death or last visit (years)

625/179

4.4 [5.3]

4.0 [5.2]

4.8 [5.3]

0.023

 Genetic background

  European

503/147

453 (90)

118 (80)

335 (94)

< 0.001

  African

503/147

50 (10)

29 (20)

21 (6)

< 0.001

Skin involvement

 lcSSc

625/179

446 (71)

 

 mRSS

342/123

9.2 ± 10.2

19.6 ± 10.1

3.5 ± 3.6

< 0.001

 Telangiectasia

572/160

264 (46)

65 (41)

199 (48)

0.112

 Calcinosis

549/152

64 (12)

7 (5)

57 (14)

< 0.001

 Digital ulcers (past or active)

538/145

161 (30)

66 (46)

95 (24)

< 0.001

Pulmonary involvement

 NYHA

    

0.702

  Classes I–II

515/150

425 (83)

122 (81)

303 (83)

 

  Classes III–IV

515/150

90 (17)

28 (19)

62 (17)

 

 6MWD (meters)

274/61

427 ± 127

432 ± 135

425 ± 125

0.705

 TLC < 70% predicted

472/145

64 (14)

33 (23)

31 (9)

< 0.001

 FVC < 70% predicted

475/148

82 (17)

44 (30)

38 (12)

< 0.001

 DLCO < 70% predicted

471/141

249 (53)

102 (72)

147 (45)

< 0.001

 Interstitial lung disease

582/166

262 (45)

115 (69)

147 (35)

< 0.001

PH (echo. and/or RHC)

547/157

67 (12)

18 (11)

49 (13)

0.775

 sPAP (echo.)

    

0.004

  < 35 mmHg

397/118

307 (77)

89 (75)

218 (78)

 

  35–46 mmHg

397/118

43 (11)

21 (18)

22 (8)

 

  > 46 mmHg

397/118

47 (12)

8 (7)

39 (14)

 

 PAH (RHC)

490/116

40 (8)

4 (3)

36 (10)

0.033

Heart involvement

 Arrhythmia

519/150

17 (3)

5 (3)

12 (3)

1.000

 AV block

512/146

7 (1)

4 (3)

3 (1)

0.106

 BB block

479/128

16 (3)

6 (5)

10 (3)

0.388

 LVEF (%)

402/102

64.9 ± 7.1

65.5 ± 8.6

64.7 ± 6.6

0.251

 Diastolic dysfunction

423/110

20 (5)

6 (5)

14 (4)

0.613

 Pericarditis

478/136

32 (7)

14 (10)

18 (5)

0.066

 Valvular disease

430/111

29 (7)

5 (5)

24 (8)

0.380

Renal involvement

 GFR < 80 ml/min

459/136

179 (39)

38 (28)

141 (44)

0.002

 Scleroderma renal crisis

428/139

44 (10)

31 (22)

13 (5)

< 0.001

Gastrointestinal involvement

611/172

429 (70)

135 (78)

294 (67)

0.006

 BMI (kg/m2)

514/159

24.4 ± 5.0

23.6 ± 4.0

24.7 ± 5.3

0.016

 Albuminemia < 35 g/l

331/108

52 (16)

28 (26)

24 (11)

< 0.001

Muscular involvement

604/172

137 (23)

71 (41)

66 (15)

< 0.001

 CPK > 200 IU/l

250/82

66 (26)

33 (40)

33 (20)

< 0.001

Joint involvement

598/172

291 (49)

127 (74)

164 (39)

< 0.001

Cancer

625/179

49 (8)

17 (10)

32 (7)

0.327

Hemoglobin

559/163

13.0 ± 1.6

12.5 ± 1.6

13.1 ± 1.5

< 0.001

Anemia

559/163

127 (23)

52 (32)

75 (19)

0.001

CRP > 8 mg/l

470/136

118 (25)

57 (42)

61 (18)

< 0.001

Serologic features

 ACA

557/151

221 (40)

6 (4)

215 (53)

< 0.001

 Anti-Scl70 antibodies

504/149

177 (35)

90 (60)

87 (25)

< 0.001

 Anti-U1RNP antibodies

342/63

15 (4)

4 (6)

11 (4)

0.492

 Anti-RNAP3 antibodies

345/72

18 (5)

13 (18)

5 (2)

< 0.001

 Anti-PMScl antibodies

343/62

16 (5)

3 (5)

13 (5)

1.000

 Anti-SSa antibodies

387/79

60 (16)

20 (25)

40 (13)

0.014

 Anti-SSb antibodies

338/62

11 (3)

3 (5)

8 (3)

0.431

 APL antibodies

441/129

31 (7)

16 (12)

15 (5)

0.007

 Low complement

482/130

18 (4)

6 (5)

12 (3)

0.589

Smoking

572/158

215 (38)

69 (44)

146 (35)

0.067

  1. Results are expressed as n (%) for qualitative variables and mean ± SD or median [IQR] for quantitative variables
  2. N number of patients with available data, lcSSc limited 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, 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