Total number of adult patients | 39 |
Median (range) age (years) | 35 (18–59) |
Gender (%) | 32 (82%) female |
Disease subtype | 39 (100%) diffuse |
Median (range) disease duration (years)a | 2 (0.5-14) |
Organ involvement: | |
Skin, n (%) | 39/39 (100%) |
mRSS units, median (range) | 24 (8–50) |
Interstitial lung disease, n (%) | 28/39 (71.79%) |
Pulmonary hypertension, n (%) | 3/39 (7.6 %) mild |
Forced vital capacity, % of predicted, mean (SD) | 74.68 (32.5) |
Heart involvement, n (%)b | 16/39 (40.9%) |
Esophageal dysmotility, n (%) | 36/39 (92.30%) |
Kidney, n | 0 |
Positive anti-Scl-70 antibodies, n (%) | 26/39 (66.6%) |
Previous immunosuppressive treatment: | |
Methotrexate, n (%) | 17/39 (43.1 %) |
Cyclophosphamide, n (%) | 29/39 (74.4 %) |
Mycophenolate mofetil, n (%) | 8/39 (20.5 %) |
Rituximab, n (%) | 2/39 (5.1 %) |
Transplant regimen: | |
Cyclophosphamide + ATG | 36/39 (92.3%) |
Fludarabine + Melphalan + ATG | 3/39 (7.7 %) |