Table 1 Patient characteristics

Age (years)

57.1 ± 13.7

56.1 ± 13.6

64.1 ± 9.1

51.3 ± 15.4

Female

104/118 (88.1%)

53/65 (81.5%)

28/29 (96.6%)

23/24 (95.8%)

Disease duration (months)

–

82 _{(42, 246)}, n = 64

80 _{(33, 142)}, n = 21

Not applicable

 lcSSc

–

34/65 (52.3%)

21/21 (100%)

Not applicable

 dSSc

–

31/65 (47.7%)

0/21 (0.0%)

Not applicable

 No skin involvement

43/118 (36.4%)

2/65 (3.1%)

19/29 (65.5%)

22/24 (91.7%)

 Only sclerodactyly

12/118 (10.2%)

4/65 (6.2%)

7/29 (24.1%)

1/24 (4.2%)

 Limited cutaneous involvement

44/118 (37.3%)

40/65 (61.5%)

3/29 (10.3%)

1/24 (4.2%)¹

 Diffuse cutaneous involvement

19/118 (16.1%)

19/65 (29.2%)

0/29 (0.0%)

0/24 (0.0%)

mRSS

2.5 _{(0, 9)}, n = 118

8.0 _{(4, 15)}, n = 65

0.0 _{(0, 2)}, n = 29

0.0 _{(0, 0)}, n = 24

Cochin Hand Function Scale

2.0 _{(0, 12)}, n = 65

4.5 _{(0, 13)}, n = 48

0.0 _{(0, 4)}, n = 21

0.0 _{(0, 4)}, n = 16

 Raynaud’s phenomenon present

115/118 (97.5%)

63/65 (96.9%)

28/29 (96.6%)

24/24 (100%)

 Puffy fingers (current)

47/101 (46.5%)

29/48 (60.4%)

17/29 (58.6%)

1/24 (4.2%)

 Digital ulcers (current)

13/118 (11.0%)

12/65 (18.5%)

1/29 (3.4%)

0/24 (0.0%)

 Digital ulcers (previously)

31/118 (26.3%)

26/65 (40.0%)

5/29 (17.2%)

0/24 (0.0%)

 Joint synovitis

24/118 (20.3%)

11/65 (16.9%)

11/29 (37.9%)

2/24 (8.3%)

 Clinical signs of arthritis

14/102 (13.7%)

4/51 (7.8%)

8/28 (28.6%)

2/23 (8.7%)

 Joint contractures

37/90 (41.1%)

33/64 (51.6%)

4/22 (18.2%)

Not available

 Tendon friction rubs

5/111 (4.3%)

4/64 (6.3%)

1/28 (3.6%)

0/24 (0.0%)

 Subcutaneous calcinosis (hands)

10/101 (9.9%)

9/50 (18.0%)

1/28 (3.6%)

0/23 (0.0%)

 ANA

116/118 (98.3%)

64/65 (98.5%)

29/29 (100%)

23/24 (95.8%)

 Anti-centromere

57/116 (49.1%)

19/63 (30.2%)

22/29 (75.9%)

16/24 (66.7%)

 Anti-Scl-70

23/118 (19.5%)

22/65 (33.8%)

1/29 (3.4%)

0/24 (0.0%)

 Anti-RNA polymerase III

12/117 (10.3%)

10/64 (15.6%)

0/29 (0.0%)

2/24 (8.3%)

 Anti-U1nRNP

2/116 (1.7%)

2/64 (3.1%)

0/28 (0.0%)

0/24 (0.0%)

 Anti-PMScl

8/111 (7.2%)

7/59 (11.9%)

0/28 (0.0%)

1/24 (4.2%)

 CRP elevation

15/117 (12.8%)

10/64 (15.6%)

3/29 (10.3%)

2/24 (8.3%)

  Dyspnoea present

53/116 (45.7%)

34/64 (53.1%)

13/29 (44.8%)

6/23 (26.1%)

  Lung fibrosis on HRCT

39/115 (33.9%)

35/62 (56.5%)

4/29 (13.8%)

0/24 (0.0%)

   DLCO (% predicted)

83.6 ± 18.9, n = 116

78.3 ± 20.0, n = 63

88.3 ± 16.6, n = 29

91.8 ± 14.3, n = 24

   FVC (% predicted)

101.8 ± 18.6, n = 117

96.1 ± 18.9, n = 64

112.1 ± 16.7, n = 29

104.5 ± 13.9, n = 24

   FEV-1 (% predicted)

95.6 ± 19.4, n = 117

90.1 ± 18.9, n = 63

103.3 ± 20.8, n = 29

100.8 ± 14.5, n = 24

   TLC (% predicted)

100.9 ± 17.5, n = 87

98.2 ± 17.7, n = 61

106.9 ± 16.7, n = 22

Not available

  Oesophageal symptoms

60/118 (50.8%)

37/65 (56.9%)

15/29 (51.7%)

8/24 (33.3%)

  Stomach symptoms

37/91 (40.7%)

26/65 (40.0%)

10/22 (45.5%)

Not available

  Intestinal symptoms

38/91 (41.8%)

27/65 (41.5%)

10/22 (45.5%)

Not available

  Palpitations

12/91 (13.2%)

9/65 (13.8%)

3/22 (13.6%)

Not available

  Conduction blocks

11/96 (11.5%)

6/47 (12.8%)

4/26 (15.4%)

1/23 (4.3%)

  LVEF (%)

63.1 ± 3.9, n = 114

63.1 ± 4.3, n = 64

63.6 ± 3.2, n = 27

62.5 ± 3.8, n = 23

 SSc pattern

90/117 (76.9%)

59/64 (92.2%)

22/29 (75.9%)

9/24 (37.5%)

Immunosuppressive therapy³

26/118 (22.0%)

21/65 (32.3%)

4/29 (13.8%)

1/24 (4.2%)