Table 2 Baseline PRESS demographic and clinical characteristics by final SSc type in the 38 patients in the at-risk population (n = 38)

 Age (years), mean (±SD) (n = 38)

45.3 (±12.8)

44.4 (±11.9)

47.5 (±14.9)

0.5129^≉

 Gender/female (n = 38)

30 (78.9)

23 (85.2)

7 (63.6)

0.1950^§

  Black

4 (10.5)

3 (11.1)

1 (9.1)

1.0000^§

  White

31 (81.6)

21 (77.8)

10 (90.9)

  Others

2 (5.3)

2 (7.4)

0 (0.0)

  Unknown

1 (2.6)

1 (3.7)

0 (0.0)

  Hispanic

4 (10.5)

4 (14.8)

0 (0.0)

0.3026^§

  Non-Hispanic

34 (89.5)

23 (85.2)

11 (100.0)

  Others/unknown

0 (0.0)

–

–

  Single

7 (18.4)

6 (22.2)

1 (9.1)

0.5506^§

  Married

29 (76.3)

20 (74.1)

9 (81.8)

  Divorced or widowed

2 (5.3)

1 (3.7)

1 (9.1)

  Others/unknown

0 (0.0)

–

–

  Full-time

25 (65.8)

20 (74.1)

5 (45.5)

0.1109^§

  Part-time

4 (10.5)

1 (3.7)

3 (27.3)

  Retired

3 (7.9)

2 (7.4)

1 (9.1)

  Disability/disabled

0 (0.0)

–

–

  Others⁺

6 (15.8)

4 (14.8)

2 (18.2)

  Never

30 (78.9)

22 (81.5)

8 (72.7)

0.6671^§

  Current or former

8 (21.1)

5 (18.5)

3 (27.3)

 Disease duration (years), mean (±SD), median (IQR)^∫ since first non-RP symptoms (n = 38)

1.0 (±0.5), 0.9 (0.7, 1.3)

1.0 (±0.5), 0.9 (0.5, 1.6)

1.0 (±0.4), 1.0 (0.8, 1.1)

0.5953^€

 Disease duration (years), mean (±SD), median (IQR) since Raynaud’s phenomenon (n=36)

3.7 (±6.4), 1.3 (0.8, 2.5)

3.6 (±6.6), 1.8 (0.8, 2.1)

4.0 (±6.2), 1.1 (0.7, 3.6)

0.9589^€

 Disease duration less than 6 months (n = 38)

4 (10.5)

4 (14.8)

0 (0.0)

0.3026^§

  Puffy hands or fingers

19 (50.0)

16 (59.3)

3 (27.3)

0.2152^¶

  Dyspnea

1 (2.6)

0 (0.0)

1 (9.1)

  Arthritis

1 (2.6)

1 (3.7)

0 (0.0)

  Reflux

1 (2.6)

0 (0.0)

1 (9.1)

  Raynaud’s phenomenon

13 (34.2)

8 (29.6)

5 (45.5)

  Skin tightening

1 (2.6)

1 (3.7)

0 (0.0)

  DU

0 (0.0)

–

–

  Others*

2 (5.3)

1 (3.7)

1 (9.1)

 Baseline mRSS (n = 34), mean (±SD)

7.4 (±4.8)

8.8 (±4.7)

4.4 (±3.8)

0.0099^≉

 Tendon friction rubs (n = 37)

7 (18.9)

7 (26.9)

0 (0.0)

0.0797^§

 Active DU (n = 37)

1 (2.7)

1 (3.8)

0 (0.0)

1.0000^§

 Calcinosis (n = 35)

4 (11.4)

3 (12.5)

1 (9.1)

1.0000^§

 ILD based on baseline HRCT (n = 16)

16 (51.6)

10 (45.5)

6 (66.7)

0.4331^§

 FVC (n = 34) (%pred), mean (±SD)

88.6 (±19.0)

86.9 (±19.4)

92.3 (±18.5)

0.4520^≉

 FVC<70% (n = 34)

8 (23.5)

5 (21.7)

3 (27.3)

1.0000^§

 DLCO (n = 33) (%pred), mean (±SD)

79.7 (±29.8)

80.0 (±26.6)

79.1 (±36.6)

0.9397^≉

 History of PH based on baseline RHC (n = 38)

1 (2.6)

1 (3.7)

0 (0.0)

1.0000^§

 Pericardial effusion on first TTE (n = 23)

2 (8.7)

2 (14.3)

0 (0.0)

0.5020^§

 LVEF of ≤ 45% on first TTE (n = 17)

0 (0.0)

–

–

–

 History of Scleroderma renal crisis (n = 38)

1 (2.6)

0 (0.0)

1 (9.1)

0.2895^§

 HAQ-DI (n = 34), mean (±SD), median (IQR)

0.7 (±0.6), 0.5 (0.3, 1.0)

0.8 (±0.7), 0.6 (0.4, 1.1)

0.4 (±0.3), 0.3 (0.3, 0.5)

0.0519^€

 ANA positive (n = 32)

32 (97.0)

23 (95.8)

9 (100.0)

1.0000^§

  Anti-Topo I (n = 30)

17 (56.7)

11 (52.4)

6 (66.7)

0.6908^§

  Anti-RNA pol III (n = 27)

10 (37.0)

6 (31.6)

4 (50.0)

0.4147^§

  Anti-U3 RNP/fibrillarin (n=7)

0 (0.0)

–

–

–

  Anti-centromere (n = 23)

0 (0.0)

–

–

–

  Anti-Th/To (n = 8)

4 (50.0)

1 (33.3)

3 (60.0)

1.0000^§

  SSA/anti-RO (n = 24)

3 (12.5)

1 (5.9)

2 (28.6)

0.1937^§

  SSB/anti-LA (n = 24)

1 (4.2)

1 (5.9)

0 (0.0)

1.0000^§

 Baseline CRP value, mean (±SD), median (IQR) (n = 22) mg/dL

2.3 (±2.7), 0.7 (0.4, 4.3)

2.2 (±2.6), 0.7 (0.4, 4.3)

3.1 (±3.4), 1.6 (0.8, 7.0)

0.2305^€

  CRP > ULN (0.6 mg/dL) (n = 22)

13 (59.1)

10 (52.6)

3 (100.0)

0.2403^§