Table 1.
Clinical and demographic characteristics of the systemic sclerosis (SSc) cohort (Charité University)
| Diffuse SSc | Limited SSc | SSc sine scleroderma | Overlap | Mixed connective tissue disease | Undifferentiated connective tissue disease | All patients | |
| Anti-topoisomerase I (LIA) | 58 (60.4) | 7 (6.2) | 1 (25.0) | 1 (2.6) | 0 | 0 | 67 (23.9) |
| Anti-topoisomerase I (ELISA) | 57 (59.4) | 6 (5.3) | 1 (25.0) | 1 (2.6) | 0 | 0 | 65 (23.2) |
| Number | 96 | 113 | 4 | 38 | 13 | 16 | 280 |
| Age (years) | 53 ± 14.5 | 60 ± 11.3 | 54 ± 12.7 | 52 ± 13.3 | 52 ± 13.5 | 56 ± 10.7 | 56 ± 13.2 |
| Duration of Raynaud's phenomenon (years) | 9 ± 10.6 | 14 ± 13.6 | 18 ± 18.47 | 8 ± 7.4 | 16 ± 10.4 | 11 ± 9.3 | 12 ± 11. 6 |
| Duration of non-Raynaud's phenomenon symptoms | 7.8 ± 1.6 | 9.5 ± 1.39 | 6 ± 10.8 | 8.8 ± 2.2 | 9.1 ± 5.5 | 9.3 ± 6.3 | 8.7 ± 7.6 |
| Duration since disease diagnosis (years) | 6 ± 7.38 | 8 ± 7.4 | 3 ± 2.6 | 6 ± 5.17 | 9.1 ± 5.5 | 7 ± 7.28 | 7 ± 7.38 |
| Female/male | 79/17 | 105/8 | 2/2 | 28/10 | 13/0 | 16/0 | 243/37 |
| mRSS | 13.2 ± 9.6 | 5.2 ± 4.2 | 1.5 ± 1.1 | 6.7 ± 7.5 | 1.6 ± 1.8 | 0.8 ± 1.1 | 7.6 ± 8.0 |
| Digital ulcers | 52 (54.2) | 38 (33.6) | 2 (50) | 14 (36.8) | 4 (30.8) | 2 (12.5) | 112 (40.0) |
| Lung fibrosis | 57 (59.4) | 16 (14.2) | 3 (75) | 16 (42.1) | 4 (30.8) | 2 (12.5) | 98 (35.0) |
| DLCO by a single breath (%) | 64.9 ± 21.9 | 76,8 ± 17.5 | 56.6 ± 12.8 | 64.2 ± 22.4 | 75.3 ± 17.8 | 75.4 ± 17.1 | 70.5 ± 20.5 |
| Mean FVC (%) | 81.7 ± 18.93 | 96.7 ± 15.2 | 87.5 ± 30.7 | 84.0 ± 19.2 | 92.9 ± 23.5 | 92 ± 18.0 | 89,2 ± 19. |
| Contractures | 78 (81.3) | 62 (54.9) | 2 (50) | 23 (60.5) | 4 (30.8) | 3 (18.8) | 172 (61.4) |
| Pulmonary arterial hypertension | 22 (22.9) | 22 (19.5) | 2 (50) | 9 (23.7) | 4 (30.8) | 1 (6.3) | 60 (21.4) |
| Renal involvement | 17 (17.7) | 22 (19.5) | 1 (25) | 10 (26.3) | 4 (30.8) | 2 (12.5) | 56 (20) |
| Renal crisis | 10 (10.4) | 3 (2.7), n = 112 | 1 (25) | 1 (2.6) | 0 | 1 (6.3) | 16 (5.7), n = 279 |
| Cardiac involvement | 47 (49.0) | 35 (31.0) | 3 (75) | 19 (50) | 6 (46.2) | 4 (25) | 117 (40.7) |
| Skin involvement | 94 (98.9) | 105 (92.9) | 3 (75) | 32 (84.2) | 7 (53.8) | 7 (43.8) | 248 (88.9) |
| Raynaud's phenomenon | 95 (99) | 110 (97.3) | 4 (100) | 38 (100) | 12 (92.3) | 13 (81.3) | 272 (97.1) |
Data presented as mean ± standard deviation or n (%). DLCO, predicted diffusion capacity; FVC, predicted forced vital capacity; LIA, line immunoblot assay; mRSS, modified Rodnan Skin Score.