Table 1 Baseline PRESS demographic and clinical characteristics in the overall population and according to baseline cutaneous subgroups (n = 301)

 Age (years), mean (±SD), (n = 301)

50.7 (±13.8)

51.5 (±13.7)

45.3 (±12.8)

0.0094^≉

 Gender/female, n (%), (n = 301)

211 (70.1)

181 (68.8)

30 (78.9)

0.2025^¶

  Black

50 (16.6)

46 (17.5)

4 (10.5)

0.5090^§

  White

222 (73.8)

191 (72.6)

31 (81.6)

  Others

24 (7.9)

22 (8.4)

2 (5.3)

  Unknown

5 (1.7)

4 (1.5)

1 (2.6)

  Hispanic

32 (10.6)

28 (10.6)

4 (10.5)

1.0000^§

  Non-Hispanic

264 (87.7)

230 (87.5)

34 (89.5)

  Others/unknown

5 (1.6)

5 (1.9)

0 (0.0)

  Single

62 (20.6)

55 (20.9)

7 (18.4)

0.5936^§

  Married

202 (67.1)

173 (65.8)

29 (76.3)

  Divorced or widowed

27 (9.0)

25 (9.5)

2 (5.3)

  Others/unknown

10 (3.3)

10 (3.8)

0 (0.0)

  Full-time

156 (51.8)

131 (49.8)

25 (65.8)

0.0239^¶

  Part-time

15 (5.0)

11 (4.2)

4 (10.5)

  Retired

49 (16.3)

46 (17.5)

3 (7.9)

  Disability/disabled

22 (6.0)

22 (6.8)

0 (0.0)

   Disabled due to scleroderma

18 (7.3)

18 (8.4)

0 (0.0)

  Others⁺

41 (13.6)

35 (13.3)

6 (15.8)

  Never

187 (62.1)

157 (59.7)

30 (78.9)

0.0222^¶

  Current or former

114 (37.8)

106 (40.3)

8 (21.1)

 Disease duration (years), mean (±SD), median (IQR)^∫ since first non-RP symptoms (n = 301)

1.2 (±0.7), 1.1 (0.7, 1.6)

1.2 (±0.7), 1.1 (0.7, 1.6)

1.0 (±0.5), 0.9 (0.7, 1.3)

0.1171^€

 Disease duration (years), mean (±SD), median (IQR) since Raynaud’s phenomenon (n = 281)

2.5 (±5.0), 1.2 (0.7, 2.0)

2.4 (±4.7), 1.2 (0.7, 2.0)

3.7 (±6.4), 1.3 (0.8, 2.5)

0.4240^€

 Disease duration less than 6 months, n (%), (n = 301)

31 (10.3)

27 (10.3)

4 (10.5)

1.0000^§

  Puffy hands or fingers

136 (45.2)

117 (44.5)

19 (50.0)

0.2270^¶

  Dyspnea

12 (4.0)

11 (4.2)

1 (2.6)

  Arthritis

19 (6.3)

18 (6.8)

1 (2.6)

  Reflux

5 (1.7)

4 (1.5)

1 (2.6)

  Raynaud’s phenomenon

64 (21.3)

51 (19.4)

13 (34.2)

  Skin tightening

38 (12.6)

37 (14.1)

1 (2.6)

  DU

4 (1.3)

4 (1.5)

0 (0.0)

  Others*

23 (7.6)

21 (8.0)

2 (5.3)

 Baseline mRSS (n = 297), mean (±SD)

21.1 (±10.2)

22.9 (±9.3)

7.4 (±4.8)

<.0001^≉

 Tendon friction rubs, n (%), (n = 285)

97 (34.0)

90 (36.3)

7 (18.9)

0.0375^¶

 Active DU, n (%), (n = 279)

17 (6.1)

16 (6.6)

1 (2.7)

0.7091^§

 Calcinosis, n (%), (n = 281)

20 (7.1)

16 (6.5)

4 (11.4)

0.2905^§

 ILD on HRCT, n (%), (n = 239)

128 (53.6)

112 (53.8)

16 (51.6)

0.8161^¶

 FVC (n = 256) (%pred), mean (±SD)

81.0 (±18.6)

79.9 (±18.3)

88.6 (±19.0)

0.0102^≉

 FVC <7 0%, n (%), (n = 256)

78 (30.5)

70 (31.5)

8 (23.5)

0.3452^¶

 DLCO (n = 243) (%pred), mean (±SD)

70.6 (±24.6)

69.3 (±23.5)

79.7 (±29.8)

0.0259^≉

 History of PH based on baseline RHC,⁺ n (%), (n = 301)

5 (1.7)

4 (1.5)

1 (2.6)

0.4932^§

 Pericardial effusion on first TTE, n (%), (n = 194)

27 (13.9)

25 (14.6)

2 (8.7)

0.7474^§

 LVEF of ≤ 45% on first TTE, n (%), (n = 138)

3 (2.2)

3 (2.5)

0 (0.0)

1.0000^§

 History of scleroderma renal crisis, n (%), (n = 301)

16 (5.3)

15 (5.7)

1 (2.6)

0.7030^§

 HAQ-DI (n = 259), mean (±SD), median (IQR)

1.1 (±0.7), 1.1 (0.5, 1.6)

1.2 (±0.7), 1.1 (0.5, 1.6)

0.7 (±0.6), 0.5 (0.3, 1.0)

0.0003^€

 ANA positive, n (%), (n = 255)

227 (89.0)

195 (87.8)

32 (97.0)

0.1438^§

  Anti-Topo I (n = 249)

72 (28.9)

55 (25.1)

17 (56.7)

0.0004^¶

  Anti-RNA pol III (n = 226)

112 (49.6)

102 (51.3)

10 (37.0)

0.1655^¶

  Anti-U3 RNP/fibrillarin (n = 64)

1 (1.6)

1 (1.8)

0 (0.0)

1.0000^§

  Anti-centromere (n = 212)

6 (2.8)

6 (3.2)

0 (0.0)

1.0000^§

  Anti-Th/To (n = 54

7 (13.0)

3 (6.5)

4 (50.0)

0.0063^§

  SSA/anti-RO (n = 201)

26 (12.9)

23 (13.0)

3 (12.5)

1.0000^§

  SSB/anti-LA (n = 201)

5 (2.5)

4 (2.3)

1 (4.2)

0.4741^§

 Baseline CRP value, mean (±SD), median (IQR) (n = 179) mg/dL

2.2 (±3.3), 0.7 (0.4, 2.7)

2.2 (±3.4), 0.7 (0.4, 2.4)

2.3 (±2.7), 0.7 (0.4, 4.3)

0.6330^€

 CRP > ULN (0.6 mg/dL), n (%), (n = 179)

98 (54.7)

85 (54.1)

13 (59.1)

0.6622^¶