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Table 2 Demographic, clinical, and immunological profile

From: Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

  Non-SScPH (n = 50) SSc-ILD-PH (n = 2) SSc-PH Group 2 (n = 2) SSc-PAH (n = 11) SSc-PAH# (Bo-PAH) (n = 5) P value Test
Age y–o (SD) 49.27 (14.4) 47 (9.8) 48.5 (12.0) 56.7 (9.7) 61.8 (9.4) 0.03 ANOVA (F = 2.99)
Female (%) 44 (88) 2 (100) 2 (100) 8 (73) 4 (80) 0.16 χ2 = 6.54
Afro-Brazilians (%) 15 (30) 2 (100) 2 (100) 6 (54) 3 (60)  < 0.0001 χ2 = 54.48
Duration in years (SD) 10.7 5 (0) 7.5 (3.5) 11.7 10.2 0.97 ANOVA (F = 0.07)
lcSSc (%) 20 (40) 0 0 5 (45) 2 (40) 0.82 χ2 = 54.48
dcSSc (%) 30 (60) 2 (100) 2 (100) 6 (54) 3 (60)  < 0.0001 χ2 = 28.62
Telangiectasia (%) 18 (36) 1 (50) 1 (50) 9 (81) 3 (60) 0.0005 χ2 = 20.05
Dyspnea (%) 9 (20) 2 (100) 1 (50) 9 (83) 4 (80)  < 0.0001 χ2 = 60.23
Urate mg/dL (SD) 4.37 (1.5) 4.3 (1.7) 4.25 (1.8) 5.58 (1.0) 4.78 (0.5) 0.03 ANOVA (F = 2.99)
Left axis deviation 1 0 0 0 1 0.78 χ2 = 0.50
FVC % (SD) 78.0 (17.0) 51.1 (2.8) 75.3 (7.0) 82.0 (13.2) 77.2 (12.4) 0.89 ANOVA (F = 0.20)
DLco% (SD) 71.6 (21.4) 25.6 (0.7) 40.2 (28) 43.7 (14.3) 43.2 (13.2)  < 0.0001 ANOVA (F = 12.91)
FVC/DLco (SD) 1.1 (0.6) 2.1 (0.9) 2.5 (2) 2.0 (0.6) 1.9 (0.4)  < 0.0001 ANOVA (F = 24.02)
Detected TRV (%) 33 (66) 2 (100) 2 (100) 11 (100) 5 (100) 0.04 χ2 = 9.92
mPAP on TTE (SD) 24.15 (5.8) 50.5 (50.9) 46.2 (22.3) 40.7 (12.3) 37.6 (10.2)  < 0.0001 ANOVA (F = 10.43)
Nucleolar ANA (%) 18 (36) 1 (50) 2 (100) 4 (36) 2 (40)  < 0.0001 χ2 = 56.55
Antibody positive (%) 18 (36) 1 (50) 0 6 (54) 2 (40) 0.19 χ2 = 4.71
Anti-Scl-70 positive (%) 9 (18) 1 (50) 0 2 (18) 1 (20)  < 0.0001 χ2 = 27.89
Anti-RNP (%) 2 (4) 0 0 1 (9) 0 0.16 χ2 = 1.93
Centromere (%) 8 (16) 0 0 3 (27) 1 (20) 0.23 χ2 = 2.95
Died (%) 4 (8) 2 (100) 2 (100) 4 (36) 3 (60)  < 0.0001 χ2 = 106.52
  1. lcSSc limited cutaneous systemic sclerosis, dcSSc diffuse cutaneous systemic sclerosis, Bo-PAH borderline pulmonary artery hypertension, FVC forced vital capacity, DLco diffusion capacity for carbon monoxide, TRV tricuspid regurgitation velocity, mPAP mean pulmonary arterial pressure, ANA antinuclear antibody, anti-Scl-70: anti-topoisomerase antibody; anti-RNP: anti-ribonucleoprotein antibody
  2. Obs.: # subgroup of all patients with SSc PAH and mPAP > 20 mmHg—< 25 mmHg, lately named bordeline (Bo-PAH)