Table 1.
Clinical and demographic characteristics of the Charité cohort
| dSSc | lSSc | SScSS | Overlap | UCTD | All | |
| Number | 96 | 113 | 4 | 51 | 16 | 280 |
| Age in yearsa | 54.5 (14.7) | 63.0 (11.2) | 52.5 (12.9) | 51 (13.2) | 55.5 (10.6) | 58.0 (13.3) |
| Duration of RP in yearsa | 5 (10.2) | 9.5 (12.6) | 12 (21.3) | 7 (8.6) | 8 (9.6) | 7.5 (11.3) |
| Duration of disease in yearsa | 5 (7.7) | 8 (7.0) | 3.5 (6.8) | 7 (7.6) | 5 (10.9) | 7 (7.6) |
| Females/males | 79/17 | 105/8 | 2/2 | 41/10 | 16/0 | 243/37 |
| mRSSa | 11.0 (9.61) | 4.0 (4.3) | 1.0 (1.1) | 3.0 (6.9) | 0.0 (1.1) | 5.0 (8.0) |
| Digital ulcersb | 52 (54.2) | 38 (33.6) | 2 (50) | 18 (35.2) | 2 (12.5) | 112 (40.0) |
| Lung fibrosisb | 57 (59.4) | 16 (14.2) | 3 (75) | 20 (39.2) | 2 (12.5) | 98 (35.0) |
| DLCO-SBa, % | 65.3 (21.9) | 78.9 (17.5) | 51.1(12.8) | 71.2 (21.7) | 80.0 (17.1) | 72.4 (20.5) |
| Mean FVCa, % | 82.1 (18.9) | 97.4 (15.2) | 95.5 (30.7) | 86.5 (20.5) | 95.2 (18.0) | 91.6 (19.0) |
| Contracturesb | 78 (81.3) | 62 (54.9) | 2 (50) | 27 (52.9) | 3 (18.8) | 172 (61.4) |
| PAHb | 22 (22.9) | 22 (19.5) | 2 (50) | 13(25.7) | 1 (6.3) | 60 (21.4) |
| Renal involvementb | 17 (17.7) | 22 (19.5) | 1 (25) | 14 (27.5) | 2 (12.5) | 56 (20.0) |
| Renal crisisb | 10 (10.4) | 3 (2.7) n = 112 |
1 (25) | 1 (2.0) | 1 (6.3) | 16 (5.7) n = 279 |
| Cardiac involvementb | 47 (49.0) | 35 (31.0) | 3 (75) | 25 (49) | 4 (25) | 117 (40.7) |
| Skin involvementb | 94 (98.9) | 105 (92.9) | 3 (75) | 39 (76.5) | 7 (43.8) | 248 (88.9) |
| RPb | 95 (99) | 110 (97.3) | 4 (100) | 50 (98) | 13 (81.3) | 272 (97.1) |
aValues displayed are medians (standard deviations). bValues displayed are numbers of patients (percentages). DLCO-SB, predicted diffusion capacity of a single breath; dSSc, diffuse systemic sclerosis; FVC, forced vital capacity; lSSc, limited systemic sclerosis; mRSS, modified Rodnan skin score; PAH, pulmonary arterial hypertension; RP, Raynaud phenomenon; SScSS, systemic sclerosis sine scleroderma; UCTD, undifferentiated connective tissue disease.