Table 1. Baseline Characteristics (Inclusion Visit) of Patients With Constant ssSSc (ie Without Skin Fibrosis Ever, at Baseline, or During Follow-up).
| Characteristics at inclusion visit | Data available, No. (n = 376) | ssSSc at inclusion visit, No. (%) (n = 376) |
|---|---|---|
| Demographics | ||
| Age, mean (SD), y | 376 | 55.3 (13.9) |
| Sex | ||
| Female | 376 | 345 (91.8) |
| Male | 376 | 31 (8.2) |
| Disease duration since first non-RP symptom, mean (SD), y | 376 | 8.3 (9.4) |
| Disease duration since RP onset, mean (SD), y | 338 | 11.9 (11.7) |
| Definition of ssSSc (adapted from Diab et al,10 2014) | 376 | 376 (100) |
| Composite definition of ssSSc (adapted from Poormoghim et al,12 2000) | 376 | 323 (85.9) |
| Disease characteristics | ||
| Skin manifestations | ||
| RP | 353 | 343 (97.2) |
| Telangiectasia | 276 | 132 (47.8) |
| Current DUs | 274 | 13 (4.7) |
| Previous DUs | 274 | 52 (19.0) |
| Current pitting scars | 269 | 31 (11.5) |
| Previous pitting scars | 269 | 31 (11.5) |
| Current puffy fingers | 365 | 147 (40.3) |
| Previous puffy fingers | 365 | 26 (7.1) |
| Other manifestations | ||
| Joint synovitis | 373 | 28 (7.5) |
| Tendon friction rubs | 369 | 5 (1.4) |
| Muscle weakness | 376 | 48 (12.8) |
| CK elevation | 280 | 16 (5.7) |
| Esophageal symptoms | 374 | 213 (57.0) |
| Stomach symptoms | 369 | 61 (16.5) |
| Intestinal symptoms | 373 | 101 (27.1) |
| History of scleroderma renal crisis | 375 | 6 (1.6) |
| Proteinuria | 313 | 16 (5.1) |
| Lung fibrosis on radiography or HRCT or presence of ILD | 327 | 129 (39.4) |
| DLCO (%pred), mean (SD) | 315 | 73.6 (21.1) |
| FVC (%pred), mean (SD) | 328 | 102.6 (21.5) |
| TLC (%pred), mean (SD) | 246 | 99.6 (20.9) |
| sPAP >40 mm Hg (echocardiography) | 259 | 20 (7.7) |
| Left ventricular ejection fraction (%), mean (SD) | 301 | 61.9 (6.2) |
| Diastolic heart dysfunction | 301 | 80 (26.6) |
| Conduction block | 274 | 25 (9.1) |
| Disease activity at baseline | ||
| EScSG disease activity index (2001), mean (SD) | 354 | 0.8 (1.0) |
| EScSG disease activity index (2016), mean (SD) | 376 | 0.4 (0.8) |
| Immunological findings | ||
| ANA+ | 373 | 360 (96.5) |
| ACA+ | 359 | 219 (61.0) |
| ATA+ | 357 | 54 (15.1) |
| RNA pol III+ | 252 | 7 (2.8) |
| PmScl+ | 236 | 12 (5.1) |
| U1RNP+ | 281 | 12 (4.3) |
| CRP >5 mg/L | 250 | 5 (2.0) |
Abbreviations: ACA, anticentromere antibodies; ANA, antinuclear antibodies; ATA, antitopoisomerase I antibodies; CK, creatine kinase; CRP, C-reactive protein; DLCO, diffusion capacities of carbon monoxide; DUs, digital ulcers; EScSG, European Systemic Sclerosis research group; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; %pred, percent predicted; RNA pol III, anti–RNA polymerase III antibodies; RP, Raynaud phenomenon; sPAP, systolic pulmonary arterial pressure; ssSSc, systemic sclerosis sine scleroderma; TLC, total lung capacities; U1RNP, anti–U1 ribonuclease protein antibodies.