Table 2.
Baseline PRESS demographic and clinical characteristics by final SSc type in the 38 patients in the at-risk population (n = 38)
| Baseline characteristics (n = total available data)∫ | Initial population at high risk of dcSSc at baseline N = 38 |
Patients who developed dcSSc during follow-up n = 27 |
Patients who did not develop dcSSc during follow-up n = 11 |
P-value£ |
|---|---|---|---|---|
| Demographic data | ||||
| 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§ |
| Race (n = 38) | ||||
| 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) | |
| Ethnicity (n = 38) | ||||
| 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) | – | – | |
| Marital status (n = 38) | ||||
| 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) | – | – | |
| Employment status (n = 38) | ||||
| 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) | |
| Smoking status (n = 38) | ||||
| Never | 30 (78.9) | 22 (81.5) | 8 (72.7) | 0.6671§ |
| Current or former | 8 (21.1) | 5 (18.5) | 3 (27.3) | |
| Clinical data | ||||
| 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§ |
| First scleroderma symptom (n = 38) | ||||
| 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€ |
| Biological data | ||||
| 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§ |
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
∫Data are expressed as n (%) unless otherwise specified; quantitative data without Gaussian distribution are presented as median (IQR) as specified
£Comparison between patients who developed dcSSc during follow-up versus those who did not
≉t-test
¶Chi-squared test
§Fisher exact test
€Wilcoxon rank sum test