Table 1:
Baseline Characteristics at PRESS Enrollment
| Overall N = 212 |
ILD N = 115 |
No ILD N = 97 |
|
|---|---|---|---|
| Age, mean ± SD years | 51.7 ± 13.8 | 53.1 ± 13.0 | 50.1 ± 14.5 |
| Female sex, n (%) | 144 (67.9) | 74 (64.3) | 70 (72.2) |
| Race, n (%) | |||
| White | 161 (75.9) | 82 (71.3) | 79 (81.4) |
| Black | 31 (14.6) | 21 (18.3) | 10 (10.3) |
| Asian | 9 (4.2) | 6 (5.2) | 3 (3.1) |
| Hispanic ethnicity, n (%) | 23 (10.8) | 13 (11.3) | 10 (10.3) |
| Smoking status, n (%) | |||
| Never | 128 (60.4) | 70 (60.9) | 58 (59.8) |
| Former | 70 (33) | 37 (32.2) | 33 (34.0) |
| Current | 14 (6.6) | 8 (7.0) | 6 (6.2) |
| Disease duration from 1st non-Raynaud’s symptom, mean ± SD years | 1.2 ± 0.7 |
1.3 ± 0.7 | 1.2 ± 0.6 |
| Modified Rodnan skin score, mean ± SD | 20.8 ± 10.3 N = 207 |
20 ± 9.9 N = 113 |
21.8 ± 10.7 N = 94 |
| Antinuclear antibody (ANA) positive, n (%) | 169 (92.3) N = 183 |
89 (90.8) N = 98 |
80 (94.1) N = 85 |
| Anti-topoisomerase I positive, n (%) | 57 (32.2) N = 177 |
40 (41.2) N = 97 |
17 (21.2) N = 80 |
| Anti-RNA polymerase III positive, n (%) | 79 (49.4) N = 160 |
37 (43.5) N = 85 |
42 (56.0) N = 75 |
| Anti-centromere positive, n (%) | 3 (2) N = 150 |
1 (1.2) N = 85 |
2 (3.1) N = 65 |
| Anti-fibrillarin positive, n (%) | 1 (2.2) N = 46 |
0 (0) N = 22 |
1 (4.2) N = 24 |
| Anti-Th/To positive, n (%) | 5 (11.4) N = 44 |
2 (8.7) N = 23 |
3 (14.3) N = 21 |
| FVC, mean ± SD liters | 3.1 ± 1.6 N = 211 |
2.9 ± 0.9 N = 114 |
3.4 ± 2.1 |
| FVC, mean ± SD % predicted | 80.4 ± 18.8 | 76.1 ± 18.7 | 85.5 ± 17.8 |
| FEV1, mean ± SD liters | 2.8 ± 5.8 N = 209 |
3.1 ± 7.8 N = 113 |
2.6 ± 0.7 N = 96 |
| FEV1, mean ± SD % predicted | 83.4 ± 19.8 N = 210 |
79.4 ± 18.9 N = 114 |
88.2 ± 19.7 N = 96 |
| FEV1/FVC ratio, mean ± SD | 84.3 ± 10.9 N = 194 |
85.3 ± 11.3 N = 107 |
83 ± 10.3 N = 87 |
| TLC, mean ± SD liters | 5 ± 3.2 N = 148 |
4.6 ± 1.2 N = 85 |
5.7 ± 4.7 N = 63 |
| TLC, mean ± SD % predicted | 86.7 ± 20.4 N = 146 |
83.6 ± 20.3 N = 85 |
91.1 ± 20.0 N = 61 |
| DLCO, mean ± SD % predicted | 68.4 ± 24.4 N = 199 |
60.5 ± 22.2 N = 109 |
78 ± 23.5 N = 90 |
| Absolute time between PFTs and HRCT, mean ± SD years | 0.3 ± 0.4 N = 211 |
0.3 ± 0.4 | 0.3 ± 0.4 N = 96 |
| Absolute time between PFTs and HRCT, median (IQR) years | 0.1 (0–0.4) N = 211 |
0.1 (0–0.4) |
0.1 (0–0.4) N = 96 |
| Mycophenolate mofetil, n (%) | 83 (39.2) | 50 (43.5) | 33 (34.0) |
| Prednisone, n (%) | 68 (32.1) | 33 (28.7) | 35 (36.1) |
| Methotrexate, n (%) | 28 (13.2) | 13 (11.3) | 15 (15.5) |
| Hydroxychloroquine, n (%) | 27 (12.7) | 15 (13) | 12 (12.4) |
| Cyclophosphamide, n (%) | 6 (2.8) | 4 (3.5) | 2 (2.1) |
| D-penicillamine, n (%) | 5 (2.4) | 2 (1.7) | 3 (3.1) |
| Azathioprine, n (%) | 4 (1.9) | 4 (3.5) | 0 (0) |
| Investigational drug, n (%) | 6 (2.8) N = 211 |
2 (1.8) N = 114 |
4 (4.1) |
| Supplemental oxygen use, n (%) | 4 (1.9) N = 211 |
3 (2.6) N = 114 |
1 (1) |
| New York Heart Association Functional Class, n (%) | N = 195 | N = 107 | N = 88 |
| I | 88 (45.1) | 43 (40.2) | 45 (51.1) |
| II | 73 (37.4) | 44 (41.1) | 29 (33) |
| III | 34 (17.4) | 20 (18.7) | 14 (15.9) |
| IV | 0 (0) | 0 (0) | 0 (0) |
| SHAQ breathlessness score, mean ± SD | 2.2 ± 2.8 N = 175 |
2.5 ± 3 N = 94 |
1.8 ± 2.5 N = 81 |
PRESS = Prospective Registry of Early Systemic Sclerosis; SD = standard deviation; FVC = forced vital capacity; FEV1 = forced expiratory volume in 1 second; TLC = total lung capacity; DLCO = diffusion capacity for carbon monoxide; IQR = interquartile range; ILD = interstitial lung disease; HRCT = high resolution computed tomography; SHAQ = Scleroderma Health Assessment Questionnaire