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Table 1 Clinical parameters of patients with limited systemic sclerosis

From: Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study

Patient

Age (years)

Sex

Disease duration (years)

Digital ulcers

PAH

mRSS/score at affected skin biopsy

ANA-ENA

1

64

F

8

no

no

10/2

Anticentromere-CenpB

2

35

F

9

no

no

09/2

Anticentromere-CenpB

3

72

F

11

yes

yes

13/2

Anticentromere-CenpB

4

49

F

7

no

no

10/2

Anticentromere-CenpB

5

37

F

5

no

no

10/2

Anticentromere-CenpB

6

52

F

10

no

no

11/2

Anticentromere-CenpB

7

59

F

8

yes

yes

13/2

Anticentromere-CenpB

8

66

F

9

no

no

09/2

Anticentromere-CenpB

9

58

F

6

no

no

10/2

Anticentromere-CenpB

10

65

F

10

no

no

10/2

Anticentromere-CenpB

Mean (SD)

55.7 (12.3)

 

8.3 (1.8)

    
  1. Abbreviations: ANA Antinuclear antibodies, ENA Extractable nuclear antigens, CenpB Centromere protein B, mRSS Modified Rodnan skin score, PAH Pulmonary arterial hypertension
  2. Patients discontinued corticosteroids, oral vasodilators, intravenous prostanoids, and other disease-modifying drugs at least 1 month before skin biopsies