Table 1.
Clinical characteristics of SSc patients of the Zurich cohort at time point of serum collection.
| Characteristics | Non-ILD SSc (n = 12) | Stable SSc-ILD (n = 12) | Progressive SSc-ILD (n = 12) | All (n = 24–36a) |
|---|---|---|---|---|
| General | ||||
| Sex, n (%) | ||||
| Female | 10 (83.3) | 10 (83.3) | 10 (83.3) | 30 (83.3) |
| Male | 2 (16.7) | 2 (16.7) | 2 (16.7) | 6 (16.7) |
| Age (mean ± SD) | 61.6 ± 9.1 | 62.0 ± 10.3 | 63.1 ± 11.1 | 61.5 ± 9.9 |
| BMI (mean ± SD) | 25.7 ± 5.8 | 24.2 ± 4.5 | 22.7 ± 4.3 | 24.2 ± 4.9 |
| SSc | ||||
| SSc disease durationb, years (mean ± SD) | 10.0 ± 10.0 | 7.1 ± 5.5 | 7.0 ± 9.9 | 9.0 ± 7.7 |
| mRSS (mean ± SD) | 3.7 ± 4.1 | 4.5 ± 5.8 | 10.3 ± 7.7 | 6.2 ± 6.6 |
| Extent of skin disease, n (%) | ||||
| dcSSc | 1 (8.3) | 2 (16.7) | 6 (50.0) | 9 (25.0) |
| lcSSc | 4 (33.3) | 2 (16.7) | 4 (33.3) | 10 (27.8) |
| Sclerodactyly only | 2 (16.7) | 4 (33.3) | 2 (16.7) | 8 (22.2) |
| No skin involvement | 5 (41.7) | 4 (33.3) | 0 (0) | 9 (25.0) |
| Joint involvement, n (%) | ||||
| Synovitis | 3 (25.0) | 0 (0) | 3 (25.0) | 6 (16.7) |
| Tendon friction rubs | 2 (16.7) | 0 (0) | 0 (0) | 2 (0.06) |
| Organ involvement, n (%) | ||||
| Cardiac diseasec | 1 (8.3) | 1 (8.3) | 1 (8.3) | 3 (8.3) |
| History of renal crisis | 0 (0) | 0 (0) | 1 (8.3) | 1 (0.03) |
| Gastrointestinal involvementc | 7 (58.3) | 4 (33.3) | 7 (58.3) | 18 (50.0) |
| Autoantibody positivity, n (%) | ||||
| Anti-centromere | 9 (75) | 2 (16.7) | 1 (8.3) | 12 (33.3) |
| Anti-topoisomerase 1 | 1 (8.3) | 2 (16.7) | 7 (58.3) | 10 (27.8) |
| Markers of systemic inflammation (mean ± SD) | ||||
| ESR, mm/h | 11.8 ± 8.4 | 25.3 ± 20.1 | 26.5 ± 20.6 | 20.9 ± 17.9 |
| CRP, mg/L | 3.1 ± 2.6 | 2.6 ± 3.5 | 4.4 ± 4.9 | 3.3 ± 3.7 |
| Disease activity index (mean ± SD) | 2.56 ± 0.92 | 2.41 ± 2.18 | 3.59 ± 2.18 | 2.91 ± 1.60 |
| Immunosuppressive treatmente, n (%) | 4 (33.3) | 7 (58.3) | 8 (66.7) | 19 (52.8) |
| ILD | ||||
| Lung involvement in CT, n (%) | ||||
| < 20% | N/A | 11 (91.7) | 8 (66.7) | 19 (79.2) |
| ≥ 20% | N/A | 2 (11.1) | 3 (25.0) | 10 (27.8) |
| ILD disease durationd, years (mean ± SD) | N/A | 1.7 ± 1.0 | 4.7 ± 2.7 | 3.2 ± 2.5 |
| Lung function parameters, % predicted (mean ± SD) | ||||
| FVC% predicted (mean ± SD) | 102.1 ± 18.8 | 108.1 ± 18.5 | 80.7 ± 22.8 | 96.9 ± 22.9 |
| DLCO% predicted (mean ± SD) | 79.2 ± 13.1 | 68.8 ± 17.7 | 58.6 ± 25.1 | 68.9 ± 20.6 |
| FEV1% predicted (mean ± SD) | 96.1 ± 19.2 | 98.3 ± 18.6 | 82.3 ± 20.7 | 92.2 ± 20.3 |
| TLC% predicted (mean ± SD) | 104.8 ± 18.6 | 108.9 ± 11.9 | 90.9 ± 23.6 | 101.5 (19.8) |
| FVC change, % (mean ± SD) | N/A | 4.4 ± 7.1 | − 14.5 ± 7.1 | − 5.1 ± 11.9 |
| DLCO change, % (mean ± SD) | N/A | 2.1 ± 14.6 | − 16.1 ± 21.6 | − 6.6 ± 20.2 |
| GAP index (mean ± SD) | N/A | 1.2 ± 1.1 | 2.6 ± 2.1 | 1.2 ± 0.5 |
| CPI index (mean ± SD) | N/A | 22.4 ± 11.5 | 38.2 ± 20.8 | 30.3 ± 18.3 |
BMI body mass index, CPI composite physiologic index, CRP C-reactive protein, dcSS diffuse cutaneous SSc, DLco carbon dioxide diffusion capacity, ESR erythrocyte sedimentation rate, FEV1 forced expiratory volume in one second, FVC forced vital capacity, lcSSc limited cutaneous SSc, GAP gender-age-physiology, mRSS modified Rodnan skin score, SD standard deviation, TLC total lung capacity.
an dependent on available datasets.
bDisease duration after onset of first non-Raynaud's symptoms, cSymptoms reported by patients
dDisease duration from first diagnosis of SSc-ILD
cIncluding treatment with following medications: azathioprine, corticosteroids, hydroxychloroquine, leflunomide, methotrexate, mycophenolate mofetil, rituximab, and tocilizumab.