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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