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Table 2 Demographics and disease characteristics of the 5 mRSS trajectories classes (n = 198)

From: Early trajectories of skin thickening are associated with severity and mortality in systemic sclerosis

 

No. with available data

Class 1

(n = 117)

Class 2

(n = 43)

Class 3

(n = 13)

Class 4

(n = 13)

Class 5

(n = 12)

p

Demographics

 Sex, female, no. (%)

198

93/117 (79.5)

28/43 (65.1)

9/13 (69.2)

7/13 (53.9)

8/12 (66.7)

0.13

 Ethnicity, no. (%)

<.001

  White

161

88/98 (89.8)

28/32 (87.5)

8/11 (72.7)

8/11 (72.7)

8/9 (88.9)

 

  Black

161

6/98 (6.1)

3/32 (9.4)

3/11 (27.3)

3/11 (27.3)

1/9 (11.1)

  Asian

161

4/98 (4.1)

1/32 (3.1)

0/11 (0.0)

0/11 (0.0)

0/9 (0.0)

 Age, mean ± SD, years

198

52.9 ± 13.9

49.2 ± 14.1

43.6 ± 17.0

47.8 ± 14.6

51.9 ± 14.5

0.15

Disease characteristics

 Disease duration, median (IQR), years

198

0.8 (0.4; 1.3)

0.9 (0.5; 1.2)

0.9 (0.5; 1.1)

0.6 (0.2; 0.9)

1.1 (0.4; 1.3)

0.21

 Duration from RP, median (IQR), years

187

1.6 (0.6; 6.0)

1.3 (0.6; 1.7)

0.8 (0.8; 2.0)

0.6 (0.2; 0.9)

1.3 (0.4; 3.3)

0.018

 Follow-up, mean ± SD, years

198

4.9 ± 0.2

5.0 ± 0.6

6.8 ± 1.0

7.4 ± 1.5

4.1 ± 0.4

0.10

 Antibodies a, no. (%)

  Anti-nuclear

165

89/93 (95.7)

37/39 (94.8)

9/11 (81.8)

11/12 (91.7)

10/10 (100)

0.32

  Anti-centromere

152

41/97 (42.3)

1/28 (3.6)

0/9 (0.0)

1/10 (10.0)

0/8 (0.0)

<.001

  Anti-topoisomerase I

152

41/97 (42.3)

21/28 (75.0)

8/9 (88.9)

8/10 (80.0)

7/8 (87.5)

<.001

  Anti-RNAP3

152

3/97 (3.1)

4/28 (14.3)

0/9 (0.0)

0/10 (0.0)

1/8 (12.5)

0.11

  Anti-U1RNP

152

7/97 (7.2)

2/28 (7.1)

0/9 (0.0)

0/10 (0.0)

0/8 (0.0)

NA

  Anti-PM/Scl

152

5/97 (5.2)

1/28 (3.6)

0/9 (0.0)

0/10 (0.0)

0/8 (0.0)

NA

  Only others

152

4/97 (4.1)

1/28 (3.6)

1/9 (11.1)

2/8 (20.0)

0/8 (0.0)

0.19

Skin variables

 Cutaneous subset, limited, no (%)

195

95/115 (82.6)

1/42 (2.4)

2/13 (15.4)

0/13 (0.0)

0/12 (0.0)

<.001

 STPR, median (IQR); units per years

198

3.9 (1.2; 9.3)

21.8 (16.0; 35.9)

7.8 (5.7; 13.4)

38.5 (29.0; 131.3)

34.4 (27.6; 74.9)

<.001

 mRSS trajectories, [95% CI]

  Mean baseline mRSS

198

4.1 [3.2; 5.0]

20.8 [19.0; 22.5]

8.7 [6.0; 11.5]

25.1 [22.6; 27.6]

35.1 [32.2; 37.9]

NA

  Mean mRSS at 6 months

198

4.3 [3.3; 5.4]

19.5 [17.7; 21.3]

14.5 [11.4; 17.6]

31.9 [28.7; 35.1]

31.1 [27.6; 34.6]

NA

  Mean mRSS at 1 year

198

4.6 [3.3; 5.9]

18.4 [16.3; 20.5]

18.7 [14.8; 22.7]

36.9 [32.7; 41.0]

27.6 [23.3; 31.8]

NA

  Mean mRSS at 2 years

198

5.1 [3.7; 6.4]

16.8 [14.5; 19.0]

23.1 [18.5; 27.6]

41.5 [37.0; 46.1]

21.5 [17.2; 25.7]

NA

  Mean mRSS at 3 years

198

5.6 [4.3; 6.9]

15.7 [13.6; 17.9]

21.6 [17.8; 25.5]

39.1 [35.3; 42.8]

16.8 [10.1; 23.5]

NA

  Mean mRSS at 4 years

198

6.2 [3.8; 8.6]

15.4 [11.0; 19.8]

14.5 [8.4; 20.7]

29.5 [22.7; 36.2]

13.5 [0; 29.5]

NA

Baseline organ involvement, no. (%)

 Telangiectasia

183

48/109 (44.0)

15/38 (39.5)

4/12 (33.3)

4/13 (30.8)

5/11 (45.5)

0.85

 Calcinosis

175

13/104 (12.5)

3/37 (8.1)

2/11 (18.2)

2/12 (16.7)

0/11 (0.0)

0.57

 Joints

191

53/114 (46.5)

31/40 (77.5)

11/13 (84.6)

10/13 (76.9)

9/11 (81.8)

<.001

 Muscles

194

20/117 (17.1)

19/40 (47.5)

5/13 (38.5)

5/13 (38.5)

4/11 (36.4)

0.001

 Digital ulcers

181

33/111 (29.7)

19/34 (55.9)

9/13 (69.2)

6/12 (50.0)

9/11 (81.8)

<.001

 Gastrointestinal tracts

187

51/111 (46.0)

25/40 (62.5)

9/12 (75.0)

9/12 (75.0)

5/12 (41.7)

0.062

 Interstitial lung disease

181

34/106 (32.1)

19/40 (47.5)

8/12 (66.7)

7/11 (63.6)

4/12 (33.3)

0.040

 FVC, median % (IQR)

160

101 (86.0; 111.0)

86.0 (70.8; 102.5)

88.5 (75;0; 111.5)

88.0 (68.5; 93.0)

82.0 (55.0; 102.0)

0.009

 DLCO, median % (IQR)

155

66.0 (50.0; 80.0)

56 (43.0; 69.0)

67.0 (53.0; 72.0)

65.0 (47.0; 86.9)

61.0 (42.0; 69.0)

0.35

 Heart

187

7/109 (6.4)

3/41 (7.3)

2/13 (15.4)

2/12 (16.7)

1/12 (8.3)

0.40

 Pulmonary hypertension

194

10/114 (8.8)

2/42 (4.8)

1/13 (7.7)

2/13 (15.4)

0/12 (0.0)

0.58

 Renal crisis

123

1/59 (1.7)

7/31 (22.6)

1/11 (9.1)

3/12 (25.0)

0/10 (0.0)

0.003

Biological variable, no. (%)

 Baseline CRP level, ≥ 6 mg/L

148

19/85 (22.4)

15/32 (46.9)

5/11 (45.5)

7/10 (70.0)

5/10 (50.0)

0.003

Treatments b, no. (%)

 Steroids and/or IS

185

57/107 (53.3)

33/40 (82.5)

13/13 (100)

12/13 (92.3)

12/12 (100.0)

<.001

  Steroids

181

54/108 (50.0)

29/38 (76.3)

11/12 (91.7)

9/12 (75.0)

9/11 (81.8)

0.002

  Methotrexate

166

13/103 (12.6)

10/32 (31.3)

3/13 (23.1)

2/9 (22.2)

4/9 (44.4)

0.028

  Azathioprine

160

6/100 (6.0)

5/31 (16.1)

2/11 (18.2)

3/9 (33.3)

3/9 (33.3)

0.008

  Mycophenolate mofetil

170

21/103 (20.4)

15/34 (44.1)

5/12 (41.7)

8/11 (72.7)

9/10 (90.0)

<.001

  Cyclophosphamide

174

18/102 (17.7)

17/36 (47.2)

6/13 (46.2)

8/12 (66.7)

6/11 (54.6)

<.001

  Rituximab

153

4/99 (4.0)

0/27 (0.0)

1/11 (9.1)

1/7 (14.3)

1/9 (11.1)

NA

  1. Anti-RNAP3 anti-RNA polymerase III antibodies, CRP C-reactive protein, disease duration duration from the first non-RP symptom, DLCO diffusing capacity of the lung for carbon monoxide (% of predicted value), FVC forced vital capacity (% of predicted value), IS immunosuppressive treatment, NA not applicable, RP Raynaud’s phenomenon, STPR skin thickening progression rate
  2. aThe sum of % may be different from 100% because some patients had either unidentified ANA or multiple autoantibodies
  3. bDuring follow-up