Table 2.
Patient characteristics
| Total | Missing data | lcSSc | dcSSc | Overlap-S. | Undiff. Scl. | Scl. sine Scl. | |
|---|---|---|---|---|---|---|---|
| Number of patients, n (%) | 1483 (100) | 0 (0) | 674 (45.5) | 484 (32.7) | 162 (10.9) | 130 (8.8) | 22 (1.5) |
| Female | 83.4 | 0.2 | 87.8 | 76.1 | 84.2 | 84.3 | 90.9 |
| Male | 16.4 | 0.2 | 12.2 | 23.9 | 15.8 | 15.7 | 9.1 |
| Family case history | 17.2 | 19.8 | 20.2 | 23.5 | 25.8 | 24.1 | 18.8 |
| ANA positive | 90.4 | 1 | 92.9 | 93.9 | 95.2 | 83.5 | 95 |
| Scl 70 positive | 27.6 | 1.2 | 16.2 | 55.8 | 15.1 | 10.7 | 40 |
| ACA positive | 36.4 | 1 | 61.5 | 11.2 | 16.4 | 34.7 | 35 |
| SSc-associated antibodies | 32.5 | 1 | 27.6 | 31.6 | 68.5 | 35.5 | 30 |
| Elevated acute-phase reactants (>30 mm/h) | 15.9 | 16.3 | 13.4 | 17.8 | 14.8 | 17.7 | 4.5 |
| Percentage of organ involvement by SSc subsets | |||||||
| RP | 94.4 | 0.1 | 96.3 | 94.2 | 95.9 | 89.3 | 95.5 |
| Skin involvement | 87.8 | 0.3 | 91.5 | 97.6 | 82.3 | 60 | 63.6 |
| PAH | 15.8 | 0.1 | 14.9 | 18.5 | 8.2 | 4.1 | 13.6 |
| Pulmonary fibrosis | 34.5 | 0.1 | 20.8 | 56.1 | 30.6 | 18.2 | 59.1 |
| Oesophagus | 60 | 0.1 | 59.2 | 69.3 | 61.2 | 35.5 | 72.7 |
| Stomach | 14.2 | 0.2 | 15.3 | 15.6 | 14.3 | 8.3 | 27.3 |
| Intestine | 5.7 | 0.2 | 6.1 | 5.3 | 5.4 | 7.4 | 18.2 |
| Kidney | 10.5 | 0.2 | 9.1 | 15.9 | 6.1 | 8.3 | 22.7 |
| Heart | 14.6 | 0.2 | 12 | 23 | 10.2 | 8.3 | 13.6 |
| Musculoskeletal system | 47.5 | 1.4 | 44.9 | 56.6 | 67.6 | 44.6 | 45.5 |
| Nervous system | 6.4 | 2.2 | 4.1 | 7.1 | 10.3 | 6.6 | 4.5 |
| Sicca-symptoms | 39.5 | 2.5 | 43.5 | 39.7 | 38.9 | 43.3 | 45.5 |
| Masticatory organ | 24.1 | 7.2 | 23.7 | 34.1 | 24.4 | 18.5 | 15 |
| Percentage of present symptoms by SSc subsets | |||||||
| Digital ulcers | 24.4 | 2.2 | 23.8 | 34.4 | 21.2 | 9.9 | 33.3 |
| Synovitis | 15.1 | 3.4 | 11.7 | 19.2 | 22.7 | 14.9 | 33.3 |
| Joint contractures | 26.1 | 3 | 22.6 | 39.7 | 21.8 | 15.7 | 22.2 |
| Tendon friction rubs | 8.3 | 3.2 | 6.6 | 13.1 | 9.9 | 8.3 | 27.8 |
| CK elevation | 9.2 | 2.8 | 5.5 | 12.1 | 22.8 | 6.7 | 11.1 |
| Muscle weakness | 27.7 | 2 | 24.7 | 33.5 | 40.4 | 22.3 | 33.3 |
| Muscle atrophy | 16.9 | 3 | 14.3 | 21.4 | 25.4 | 9.1 | 16.7 |
| Dysphagia, reflux | 60.4 | 1.9 | 60.8 | 69.2 | 63 | 43 | 61.1 |
| Satiety, nausea | 16.5 | 3.6 | 17.9 | 16.4 | 20.6 | 19.8 | 33.3 |
| Diarrhoea, constipation | 24.2 | 3.6 | 27.2 | 25.2 | 22.1 | 32.2 | 35.3 |
| Hypertension | 24 | 2.2 | 28.2 | 24.5 | 19.9 | 29.8 | 38.9 |
| Renal insufficiency | 13.7 | 2.6 | 16.5 | 15.6 | 8.3 | 8.3 | 22.2 |
| Proteinuria | 9.5 | 3.1 | 8.9 | 15.2 | 9.7 | 6.7 | 11.1 |
| Dyspnoea | 31.2 | 2 | 32 | 40.4 | 23.3 | 18.2 | 50 |
| Palpitations | 22.3 | 2.8 | 23.1 | 20.1 | 21.5 | 23.1 | 27.8 |
| Conduction block | 12.9 | 2.6 | 10.8 | 19.2 | 10.3 | 8.3 | 16.7 |
| Diastolic dysfunction | 14.1 | 3.4 | 14.9 | 19 | 9.1 | 8.3 | 17.6 |
| Lung restrictive disease | 23.7 | 2.8 | 16.1 | 40.7 | 18.6 | 10 | 33.3 |
| Polyneuropathy | 8 | 4.9 | 6.8 | 8 | 8.8 | 5.9 | 0 |
| Trigeminal neuralgia | 1.6 | 4.7 | 1.1 | 2.6 | 1.5 | 1.7 | 0 |
| DLCO (≤ 75% | 24.8 | 51.9 | 23.6 | 31.4 | 21 | 10.8 | 9.1 |
| mRSS (mean ± s.d.) | 9.2 ± 9.2 | 13.1 | 6.9 ± 6.2 | 15.2 ± 10.9 | 6.9 ± 7.9 | 2.7 ± 4.3 | 0.6 ± 0.8 |