Table 1

Clinical and demographical features of the BIOPSY cohort

Early dcSSc at baselineEarly dcSSc at 12 monthsEstablished dcSSclcSScHC
Total (n)2120151616
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)
MRSS18 (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 (%)00010 (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)01 (6.3)
 PM/DM (%)6 (28.6)6 (30)3 (20)0
 Sjogren’s (%)001 (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)000
 Tocilizumab (%)1 (4.8)3 (15)00
 Cyclophosphamide (%)1 (4.8)000
 IvIG (%)02 (10)1 (6.7)0
 Untreated (%)3 (14.3)05 (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.