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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