Table 1 Main demographic data and clinical and laboratory features composing the European Scleroderma Study Group index among patients enrolled in both the internal (Milan) and external (Naples) cohorts

Number of patients

122

97

219

Male/female ratio

8/114

9/88

17/202

Median age, years (range)

52 (17–82)

55 (19–79)

54 (17–82)

Median disease duration, years (range)

4 (0–28)

6 (0–36)

5 (0–36)

lcSSc/dcSSc

60/62

72/25

132/87

 ACAs

57

53

110

 Anti-Scl-70

58

22

80

 Others

7

22

29

ESSG index ≥3, n (%)

57 (46.8)

30 (30.9)

87 (39.7)

ESSG index ≥3.5, n (%)

49 (40.2)

20 (20.6)

69 (31.5)

Mean mRSS (range)

5.9 (0–22)

3.4 (0–23)

4.6 (0–23)

Scleroderma, n (%)

74 (60.7)

17 (17.5)

91 (41.5)

Change in skin involvement^a, n (%)

35 (28.7)

23 (23.7)

58 (26.4)

Ulcers, n (%)

34 (27.9)

11 (11.3)

45 (20.5)

Change in vascular features^a,b, n (%)

56 (46.7)

19 (19.5)

75 (34.2)

Arthritis, n (%)

30 (24.6)

7 (7.2)

37 (16.8)

DLCO <80%, n (%)

86 (71.3)

85 (87.6)

171 (78)

Change in cardiopulmonary features^a,c, n (%)

25 (20.5)

19 (19.5)

44 (20)

ESR >30 mm/h, n (%)

33 (27)

35 (36)

68 (31.1)

Low complement, n (%)

15 (12.3)

13 (13.4)

28 (12.7)