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Table 1 Clinical and demographic features of the 10 diffuse systemic sclerosis patients

From: Macitentan inhibits the transforming growth factor-β profibrotic action, blocking the signaling mediated by the ETR/TβRI complex in systemic sclerosis dermal fibroblasts

Sex/age (years)

Year of SSc onset/disease duration at skin biopsy (years)

MRSS/score at skin biopsy

Autoantibodies

Lung involvement HRCT/PFT

Heart involvement/Scleroderma renal crisis

Raynaud’s phenomenon/digital ulcers

F/46

2010/2

12/2

ANA/Scl-70

Normal/Normal

Normal/No

Yes/No

F/21

2009/3

13/1

ANA/Scl-70

Normal/Normal

Normal/No

Yes/Yes

F/31

2011/1

13/2

ANA/Scl-70

Normal/Normal

Normal/No

Yes/Yes

F/36

2010/2

11/2

ANA/Scl-70

Normal/Normal

PAH/No

Yes/Yes

M/20

2010/2

11/1

ANA/Scl-70

Normal/Normal

Normal/No

Yes/No

F/41

2010/2

15/2

ANA/Scl-70

Normal/Normal

Normal/No

No/No

F/30

2010/2

10/1

ANA/Scl-70

Normal/Normal

Normal/No

Yes/No

F/21

2010/2

09/1

ANA/Scl-70

Normal/Normal

Normal/No

Yes/No

F/31

2009/3

14/1

ANA/Scl-70

Normal/Normal

Normal/No

Yes/No

F/42

2009/3

16/2

ANA/Scl-70

Fibrosis/Normal

Normal/No

Yes/No

  1. The internal organ involvement refers to the time of biopsy. ANA antinuclear antibodies, F female, HRCT high-resolution computed tomography, M male, MRSS modified Rodnan skin thickness score (maximum possible score 51), PAH pulmonary arterial hypertension, PFT pulmonary function test, Scl-70 anti-topoisomerase, SSc systemic sclerosis