Table 1 Patient and healthy donor characteristics

Mean age, years (SD)

55 (13)

43 (7)

Females/males, n (%)

24/9 (73/27)

9/4 (69/31)

Mean anti-AT₁R Abs, units (SD)

16 (8)

9 (4)

Mean anti-ET_AR Abs, units (SD)

15 (10)

6 (4)

Diffuse cutaneous form, limited cutaneous form, other, n (%)

21 (64), 10 (30), 2 (6)

n.a.

Scl 70 positive, n (%), n.d. n (%)

13 (39), 3 (90)

n.a.

Anti-centromere positive, n (%), n.d. n (%)

4 (12), 4 (12)

n.a.

Duration since Raynaud phenomenon, years (±SD)

10 (11)

n.a.

Duration since skin-involvement onset, years (±SD)

9 (7)

n.a.

Duration since internal organ onset, years (±SD)

8 (7)

n.a.

mRSS median (IQR)

8 (4-18)

n.a.

Pulmonary arterial hypertension, n (%)^a

14 (42)

n.a.

Lung fibrosis, n (%)^b

18 (55)

n.a.

Mean DLCO% (SD)

50 (20)

n.a.

Mean FVC% (SD)

85 (18)

n.a.

Animal experiments

SSc patients (n = 14)

Healthy donors (n = 15)

Mean age, years (SD)

57 (14)

47 (8)

Females/males, n (%)

11/3 (79/21)

12/3 (80/20)

Mean anti-AT₁R Abs, units (SD)

18 (10)

6 (4)

Mean anti-ET_AR Abs, units (SD)

17 (10)

4 (3)

Diffuse cutaneous form, limited cutaneous form, other, n (%)

10 (71), 3 (21), 0 (0)

n.a.

Scl 70 positive, n (%), n.d. n (%)

10 (71), 1 (7)

n.a.

Anti-centromere positive, n (%), n.d. n (%)

2 (14), 3 (21)

n.a.

Duration since Raynaud phenomenon, years (±SD)

11 (8)

n.a.

Duration since skin-involvement onset, years (±SD)

10 (7)

n.a.

Duration since internal-organ onset, years (±SD)

10 (9)

n.a.

mRSS median (IQR)

8.5 (5.3-11)

n.a.

Pulmonary arterial hypertension, n (%)^a

8 (57)

n.a.

Lung fibrosis, n (%)^b

8 (57)

n.a.

Mean DLCO% (SD)

42 (18)

n.a.

Mean FVC% (SD)

77 (19)

n.a.