Table 1 Clinical and demographic characteristics of the systemic sclerosis (SSc) cohort (Charité University)

Anti-topoisomerase I (LIA)

58 (60.4)

7 (6.2)

1 (25.0)

1 (2.6)

0

0

67 (23.9)

Anti-topoisomerase I (ELISA)

57 (59.4)

6 (5.3)

1 (25.0)

1 (2.6)

0

0

65 (23.2)

Number

96

113

4

38

13

16

280

Age (years)

53 ± 14.5

60 ± 11.3

54 ± 12.7

52 ± 13.3

52 ± 13.5

56 ± 10.7

56 ± 13.2

Duration of Raynaud's phenomenon (years)

9 ± 10.6

14 ± 13.6

18 ± 18.47

8 ± 7.4

16 ± 10.4

11 ± 9.3

12 ± 11. 6

Duration of non-Raynaud's phenomenon symptoms

7.8 ± 1.6

9.5 ± 1.39

6 ± 10.8

8.8 ± 2.2

9.1 ± 5.5

9.3 ± 6.3

8.7 ± 7.6

Duration since disease diagnosis (years)

6 ± 7.38

8 ± 7.4

3 ± 2.6

6 ± 5.17

9.1 ± 5.5

7 ± 7.28

7 ± 7.38

Female/male

79/17

105/8

2/2

28/10

13/0

16/0

243/37

mRSS

13.2 ± 9.6

5.2 ± 4.2

1.5 ± 1.1

6.7 ± 7.5

1.6 ± 1.8

0.8 ± 1.1

7.6 ± 8.0

Digital ulcers

52 (54.2)

38 (33.6)

2 (50)

14 (36.8)

4 (30.8)

2 (12.5)

112 (40.0)

Lung fibrosis

57 (59.4)

16 (14.2)

3 (75)

16 (42.1)

4 (30.8)

2 (12.5)

98 (35.0)

DLCO by a single breath (%)

64.9 ± 21.9

76,8 ± 17.5

56.6 ± 12.8

64.2 ± 22.4

75.3 ± 17.8

75.4 ± 17.1

70.5 ± 20.5

Mean FVC (%)

81.7 ± 18.93

96.7 ± 15.2

87.5 ± 30.7

84.0 ± 19.2

92.9 ± 23.5

92 ± 18.0

89,2 ± 19.

Contractures

78 (81.3)

62 (54.9)

2 (50)

23 (60.5)

4 (30.8)

3 (18.8)

172 (61.4)

Pulmonary arterial hypertension

22 (22.9)

22 (19.5)

2 (50)

9 (23.7)

4 (30.8)

1 (6.3)

60 (21.4)

Renal involvement

17 (17.7)

22 (19.5)

1 (25)

10 (26.3)

4 (30.8)

2 (12.5)

56 (20)

Renal crisis

10 (10.4)

3 (2.7), n = 112

1 (25)

1 (2.6)

0

1 (6.3)

16 (5.7), n = 279

Cardiac involvement

47 (49.0)

35 (31.0)

3 (75)

19 (50)

6 (46.2)

4 (25)

117 (40.7)

Skin involvement

94 (98.9)

105 (92.9)

3 (75)

32 (84.2)

7 (53.8)

7 (43.8)

248 (88.9)

Raynaud's phenomenon

95 (99)

110 (97.3)

4 (100)

38 (100)

12 (92.3)

13 (81.3)

272 (97.1)