Table 1.
Baseline PRESS demographic and clinical characteristics in the overall population and according to baseline cutaneous subgroups (n = 301)
| Baseline characteristics (n = total available data)∫ | Overall population N = 301 |
Definite dcSSc n = 263 |
At risk for dcSSc n = 38 |
P-value£ |
|---|---|---|---|---|
| Demographic data | ||||
| 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¶ |
| Race, n (%), (n = 301) | ||||
| 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) | |
| Ethnicity, n (%), (n = 301) | ||||
| 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) | |
| Marital status, n (%), (n = 301) | ||||
| 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) | |
| Employment status, n (%), (n = 301) | ||||
| 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) | |
| Smoking status, n (%), (n = 301) | ||||
| Never | 187 (62.1) | 157 (59.7) | 30 (78.9) | 0.0222¶ |
| Current or former | 114 (37.8) | 106 (40.3) | 8 (21.1) | |
| Clinical data | ||||
| 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§ |
| First scleroderma symptom, n (%), (n = 301) | ||||
| 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€ |
| Biological data | ||||
| 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¶ |
mRSS, modified Rodnan skin score; FVC, forced vital capacity; DLCO, diffusion capacity for carbon monoxide; RHC, right heart catheterization; LVEF, left ventricular ejection fraction; TTE, transthoracic echocardiogram; ILD, interstitial lung disease; Topo I, topoisomerase I; RNA pol III, RNA polymerase III; CRP, C-reactive protein; HAQ-DI, Health Assessment Questionnaire-Disability Index; IQR, inter-quartile range; SD, standard deviation; ULN, upper limit of normal
*Other first scleroderma symptom includes lower extremity swelling, telangiectasias, wrist and ankle inflammation, joint pain, fatigue, myalgias, Carpal tunnel syndrome, cold and numbness in extremities, pruritis, hypo/hyper-pigmentation, hypertension, cough, and gastrointestinal discomfort
+Based on the results of n = 22 RHC on 22 participants
∫Data are expressed as n (%) unless otherwise specified; quantitative data without Gaussian distribution are presented as median (IQR) as specified
£Comparison between definite dcSSc and high-risk population at baseline
≉t-test
¶Chi-squared test
§Fisher exact test
€Wilcoxon rank sum test