Table 1.
Clinical characteristics at baseline (study-1).
| SSc patients without-ILD (N = 124) | SSc patients with-ILD (N = 41) | HC (N = 13) | |
|---|---|---|---|
| Age in years, median (IQR) | 61 (54–72) | 65 (53–72) | 53 (47–57) |
| Female, N (%) | 102 (82.3) | 28 (68.3) | 8 (61.5) |
| Extent of skin involvement, n (%): | |||
| LcSSc | 114 (91.9) | 35 (85.4) | |
| dcSSc | 9 (7.3) | 6 (14.6) | |
| Sine scleroderma | 1 (0.8) | 0 (0.0) | |
| Puffy fingers or sclerodactyly, n (%) | 103 (83.1) | 35 (85.4) | |
| Pitting scars or digital ulcers, n (%) | 66 (53.2) | 17 (41.5) | |
| Telangiectasia, n (%) | 83 (74.1) (ND: 12) | 29 (78.4) (ND: 4) | |
| Raynaud's phenomenon, n (%) | 124 (100.0) | 40 (97.6) | |
| Autoantibody profile, n (%): | |||
| Anti-nucleolar antibody | 29 (23.4) | 22 (53.7) | |
| Anti-centromere | 74 (59.7) | 8 (19.5) | |
| Anti-topoisomerase I | 10 (8.1) | 8 (19.5) | |
| Anti-RNA polymerase 3 | 1 (0.8) | 0 (0.0) | |
| Other | 10 (8.1) | 3 (7.3) | |
| Calcinosis cutis, n (%) | 40 (36.4) (ND: 14) | 11 (32.4) (ND: 7) | |
| Gastrointestinal involvement, n (%) | 88 (73.9) (ND: 5) | 25 (62.5) (ND: 1) | |
| HRCT Pattern, (n) | |||
| iNSIP | – | 7 | |
| fNSIP | – | 26 | |
| UIP | – | 6 | |
| Other | – | 2 | |
| PFTs, median (IQR): | |||
| % FVC | 109.0 (95.5–120.0) (ND: 15) | 94.5 (68.8–107.0) (ND: 3) | |
| % DLCO | 74.0 (62.0–83.5) (ND: 15) | 54.0 (42.5–70.5) (ND: 9) | |
| COPD, n (%) | 28 (22.6) | 6 (14.6) | |
| Immunosuppression | 17 (13.7) | 15 (36.6) | |
| Vasodilators | 16 (12.9) | 3 (7.3) | |
| Glucocorticoids | 10 (8.1) | 7 (17.1) |
lcSSc: limited cutaneous SSc; dcSSc: Diffuse cutaneous SSc; FVC: forced vital capacity, DLco: capacity for diffusion of carbon monoxide, COPD: chronic obstructive pulmonary disease; ND: not documented; iNSIP: inflammatory nonspecific interstitial pneumonia; fNSIP: fibrotic NSIP; UIP: usual interstitial pneumonia.