Baseline cohort characteristics of patients with dcSSc
| Variable | Value | Missingness of the variable |
| Total number of patients with dcSSc | 152 | NA |
| Age, years, median (Q1–Q3) | 54.00 (44.0–61.0) | 0% |
| Male gender, n (%) | 44 (28.9) | 0% |
| Time since RP onset, years, median(Q1–Q3) | 8.0 (4.0–15.0) | 17% |
| Disease duration*, years, median(Q1–Q3) | 7.0 (3.2–12.0) | 1% |
| Disease duration <3 years | 23 | 1% |
| ANA positive, n (%) | 102 (94.4) | 13% |
| ACA positive, n (%) | 5 (4.9) | 13% |
| Scl-70 positive, n (%) | 59 (55.7) | 13% |
| Anti-RNA polymerase III positive, n (%) | 15 (16.7) | 15% |
| mRSS, median(Q1–Q3) | 9.0 (5.0–15.0) | 26% |
| Presence of RP, n (%) | 123 (96.1) | 16% |
| Digital ulcers—current, n (%) | 23 (18.5) | 9% |
| Digital ulcers—never, n (%) | 63 (50.8) | 9% |
| Joint contractures, n (%) | 68 (55.7) | 20% |
| Joint synovitis, n (%) | 2 (1.6) | 20% |
| Oesophageal symptoms (dysphagia, reflux), n (%) | 91 (65.9) | 9% |
| Stomach symptoms (early satiety, vomiting), n (%) | 27 (22.0) | 19% |
| Intestinal symptoms (diarrhoea, bloating, constipation), n (%) | 54 (38.8) | 9% |
| Malabsorption syndrome, n (%) | 10 (10.8) | 39% |
| Dyspnoea, NYHA stages I and II, n (%) | 91 (86.7) | 27% |
| Dyspnoea, NYHA stages III and IV, n (%) | 14 (13.3) | 27% |
| Pulmonary hypertension, n (%) | 10 (10.6) | 20% |
| Lung fibrosis detected by HRCT, n (%) | 38 (67.9) | 27% |
| FVC, % predicted, median (IQR) | 87(75.5, 98.0) | 41% |
| DLCO/SB, % predicted, median (Q1–Q3) | 57.00 (48.5–71.5) | 43% |
| Immunosuppression, n (%) | 33 (32.7) | 34% |
| ESR >25 mm/hour, n (%) | 82 (68.3) | 21% |
| CRP elevation, n (%) | 31 (28.7) | 29% |
| ScleroID score, median (Q1–Q3) | 3.22 (1.7–4.7) | 2% |
| Self-reported current disease status† Very good Good Acceptable Bad Very bad | 14 (9.2%) 38 (25.0%) 75 (49.3%) 19 (12.5%) 5 (3.3%) | 0% |
| Patients’ global assessment | 4 (2–6) | 1% |
*Disease duration was defined as the time since the first non-RP manifestation.
†The self-reported current disease status was assessed by the following question in the case report form: Think about all the ways in which the systemic sclerosis has affected you during the last week, how would you consider this state?
ACA, anticentromere antibodies; ANA, antinuclear antibodies; CRP, C reactive protein; dcSSc, diffuse cutaneous systemic sclerosis; DLCO/SB, diffusing capacity of the lung for carbon monoxide/single breath; ESR, erythrocyte sedimentation rate; FVC, forced vital capacity; HRCT, high-resolution CT; mRSS, modified Rodnan Skin Score; NA, not applicable; NYHA, New York Heart Association; RP, Raynaud’s phenomenon; ScleroID, Systemic sclerosis Impact of Disease.