Clinical and demographical features of the BIOPSY cohort
| Early dcSSc at baseline | Early dcSSc at 12 months | Established dcSSc | lcSSc | HC | |
| Total (n) | 21 | 20 | 15 | 16 | 16 |
| F (%) | 12 (57.1) | 11 (55) | 12 (80) | 12 (75) | 9 (56.3) |
| Age (yrs) | 52 (23–75) | 54 (24–76) | 56.9 (24–73) | 52.5 (27–85) | 43.3 (28–81) |
| Disease duration (yrs) | 1.75 (0.5–4.9) | 2.6 (1.5–5.9) | 13 (5–20.8) | 9 (3.5–30.4) | |
| MRSS | 18 (7–39) | 16 (2–38) | 10 (2–36) | 4 (2–12) | |
| Antibody | |||||
| ATA (%) | 8 (38.1) | 7 (35) | 4 (26.7) | 2 (12.5) | |
| ARA (%) | 6 (28.6) | 6 (30) | 6 (40) | 0 | |
| ACA (%) | 0 | 0 | 0 | 10 (62.5) | |
| ANA neg (%) | 2 (9.5) | 2 (10) | 1 (6.7) | 1 (6.3) | |
| Others (%) | 5 (23.8) | 5 (25) | 7 (46.7) | 3 (18.8) | |
| Organ involvement | |||||
| Lung (%) | 6 (28.6) | 7 (35) | 8 (53.3) | 0 | |
| Muscle (%) | 6 (28.6) | 6 (30) | 1 (6.7) | 0 | |
| Kidney (%) | 3 (14.3) | 4 (20) | 1 (6.7) | 0 | |
| PAH (%) | 1 (4.8) | 1 (5) | 1 (6.7) | 0 | |
| Cardiac (%) | 3 (14.3) | 3 (15) | 1 (6.7) | 1 (6.3) | |
| GI (%) | 3 (14.3) | 3 (15) | 4 (26.7) | 1 (6.3) | |
| Overlap conditions | |||||
| RA (%) | 1 (4.8) | 3 (15) | 0 | 1 (6.3) | |
| PM/DM (%) | 6 (28.6) | 6 (30) | 3 (20) | 0 | |
| Sjogren’s (%) | 0 | 0 | 1 (6.7) | 2 (12.5) | |
| Immunosuppression | |||||
| MMF (%) | 9 (42.9) | 17 (85) | 9 (60) | 0 | |
| MTX (%) | 7 (33.3) | 5 (25) | 2 (13.3) | 3 (18.8) | |
| HCQ (%) | 5 (23.8) | 5 (25) | 1 (6.7) | 5 (31.3) | |
| Azathioprine (%) | 1 (4.8) | 0 | 0 | 0 | |
| Tocilizumab (%) | 1 (4.8) | 3 (15) | 0 | 0 | |
| Cyclophosphamide (%) | 1 (4.8) | 0 | 0 | 0 | |
| IvIG (%) | 0 | 2 (10) | 1 (6.7) | 0 | |
| Untreated (%) | 3 (14.3) | 0 | 5 (33.3) | 9 (56.2) | |
Results presented as median and range unless otherwise stated.
ACA, anticentromere antibody; ANA, antinuclear autoantibody; ARA, anti-RNA polymerase III antibody; ATA, antitopoisomerase-1 antibody; BIOPSY, BIOlogical Phenotyping of diffuse SYstemic sclerosis; DM, dermatomyositis; dsSSc, diffuse cutaneous systemic sclerosis; F, female; GI, gastrointestinal; HC, healthy volunteer; HCQ, hydroxychloroquine; IvIG, intravenous immunoglobulin; lcSSc, limited cutaneous systemic sclerosis; MMF, mycophenolate mofetil; MRSS, Modified Rodnan Skin Score; MTX, methotrexate; PAH, pulmonary arterial hypertension; PM, polymyositis; RA, rheumatoid arthritis.