Baseline characteristics of the systemic sclerosis population with a baseline visit and 1 year follow-up*
| Patients | DcSSc | LcSSc | LSSc | |
|---|---|---|---|---|
| N=171 | N=64 | N=79 | N=28 | |
| Sociodemographics | ||||
| Age, years, mean (SD) | 53.2 (14.3) | 50 (14) | 56.2 (13.6) | 51.8 (15.8) |
| Female, N (%) | 135 (78.9) | 44 (68.8) | 65 (82.3) | 26 (92.9) |
| Caucasian origin, N (%)† | 118 (69) | 44 (68.8) | 54 (68.4) | 20 (71.4) |
| Disease characteristics, N (%) | ||||
| Disease duration, years, median (IQR) | 2 (0–10) | 3 (1–8) | 4 (0.8–12) | 0 (0–1) |
| DU | 43 (25.1) | 14 (21.9) | 24 (30.4) | 5 (17.9) |
| Telangiectasia | 100 (58.5) | 32 (50) | 55 (69.6) | 13 (46.4) |
| Synovitis | 18 (10.5) | 6 (9.4) | 9 (11.4) | 3 (10.7) |
| Friction rubs | 6 (3.5) | 5 (7.8) | 1 (1.3) | 0 (0) |
| Calcinosis | 26 (15.2) | 6 (9.4) | 16 (20.3) | 4 (14.3) |
| Proximal muscular weakness | 9 (5.3) | 8 (12.5) | 1 (1.3) | 0 (0) |
| Renal crisis | 6 (3.5) | 3 (4.7) | 3 (3.8) | 0 (0) |
| MRSS, median (IQR) | 2.5 (0–6) | 6 (2–19) | 2.5 (2–4) | 0 (0–0) |
| Autoantibodies, N (%) | ||||
| ANA‡ | 155 (90.6) | 56 (87.5) | 75 (94.9) | 24 (85.7) |
| Anti-Scl-70† | 39 (22.8) | 28 (43.8) | 10 (12.7) | 1 (3.6) |
| Anticentromere‡ | 64 (37.4) | 4 (6.3) | 40 (50.6) | 20 (71.4) |
| Cardiopulmonary investigations | ||||
| FVC % of predicted, mean (SD)§ | 99.1 (22.7) | 88.4 (20.7) | 103.4 (21.1) | 110.8 (22.4) |
| DLCO % of predicted, mean (SD)§ | 64 (17.6) | 59.7 (18.6) | 64.3 (16) | 72.9 (17) |
| ILD, N (%) | 82 (48) | 40 (62.5) | 36 (45.6) | 6 (21.4) |
| SPAP≥35 mm Hg, N (%) | 28 (16.4) | 12 (18.8) | 14 (17.7) | 2 (7.1) |
| LVEF %, mean (SD) | 60.3 (7.7) | 60.8 (9) | 59.9 (6.7) | 60.5 (56–65.8) |
| Pericardial fluid, N (%) | 5 (2.9) | 3 (4.7) | 1 (1.3) | 1 (3.6) |
| SSc pattern on capillaroscopy¶ | 52 (94.5) | 9 (90) | 28 (96.6) | 15 (88.2) |
| Immunosuppressive therapy, N (%) | ||||
| Current | 55 (32.2) | 22 (34.4) | 24 (30.4) | 9 (32.1) |
| Previous | 60 (35.1) | 38 (59.4) | 19 (24.1) | 3 (10.7) |
| SHAQ (0–3), median (IQR)‡ | 0.63 (0.13–1.00) | 0.63 (0.22–1.13) | 0.5 (0.13–0.88) | 0.63 (0.13–0.88) |
| SF-36, median (IQR)† | ||||
| Physical component summary scale | 39.7 (31.5–48.7) | 41.4 (28.4–49.6) | 39.6 (32.5–48) | 39.1 (31.8–49.5) |
| Mental component summary scale | 52.5 (41–56.6) | 53.2 (46–58.4) | 51.3 (38.8–55) | 51.5 (37.7–57) |
*Including 8 patients who died within 1 year of follow-up.
†<15% missing.
‡<10% missing.
§<5% missing.
¶Capillaroscopy is performed in 55 patients (32%) at baseline.
ANA, antinuclear antibody; Anti-Scl-70, anti-topoisomerase; DcSSc, diffuse cutaneous SSc; DLCO, diffusing capacity for carbon monoxide; DU, digital ulcers; FVC, forced vital capacity; ILD, interstitial lung disease; LcSSC, limited cutaneous SSc; LSSC, limited non-cutaneous SSc; LVEF, left ventricle ejection fraction; MRSS, modified Rodnan Skin Score; SF-36, Short-Form-36; SHAQ, Scleroderma Health Assessment Questionnaire; SPAP, systolic pulmonary arterial pressure.