Table 1.

Data of patients with SSc according to the presence of pulmonary disease.

 Pulmonary Disease, n = 13No Pulmonary Disease, n = 16P
Age, yrs50 (44–62)53 (42–63)0.82
Women, n (%)13 (100)15 (93)> 0.99
Disease duration, yrs8 (3–10)5 (2–8)0.48
Systemic hypertension, n (%)5 (38)1 (6)0.06
Diabetes mellitus, n (%)3 (23)1 (6)0.30
Sjögren syndrome, n (%)4 (30)1 (6)0.14
Cutaneous involvement, n (%)0.06
      dcSSc7 (54)3 (18)
      lcSSc6 (46)13 (82) 
Drug therapies, n (%)
      Mycophenolate mofetil8 (62)2 (13)0.02
      Methotrexate3 (23)4 (25)> 0.99
      Calcium channel blockers8 (62)5 (31)0.14
      PDE5 inhibitors3 (23)00.08
      ET-1 receptor inhibitors2 (15)00.19
Pulmonary involvement, n (%)
      Pulmonary hypertension5 (38) 
      Interstitial lung disease4 (31) 
      Both4 (31) 
Raynaud phenomenon, n (%)10 (77)11 (69)0.70
Modified Rodnan skin score17 (12–21)10 (7–14)0.02
Valentini Disease Activity Index3 (1–3.5)0.5 (0–1.2)0.002
Medsger Disease Severity Scale6 (6–8)2 (1–3)< 0.001
NYHA functional class, n (%)0.03
      Class I9 (69)16 (100)
      Class ≥ II4 (31)0 
Autoantibodies, n (%)
      ANA+13 (100)16 (100)> 0.99
      CENP-B+5 (38)11 (68)0.14
      Scl-70+3 (23)00.08
CRP, mg/L3.1 (1.2–6.8)2.0 (0.9–3.4)0.33
ESR, mm/h30 (18–38)10 (4–16)0.02
25(OH)D, n (%)0.21
      Normal (≥ 30 ng/mL)02 (13)
      Insufficient (≥ 20 to < 30 ng/mL)3 (23)1 (6) 
      Deficient (< 20 ng/mL)10 (77)13 (81) 
Echocardiographic findings
      LVEF, %64 (60–66)64 (60–65)0.78
      PASP, mmHg35 (3–68)29 (25–36)0.02
      TAPSE, mm22 (14–25)22 (20–23)0.71
  • Data are presented as median (IQR) unless otherwise specified. Discrete variables were evaluated using the chi-square or Fisher exact test, as appropriate; continuous variables were evaluated using the Mann-Whitney U test. Significant P values are in bold. 25(OH)D: 25-hydroxyvitamin D; ANA: antinuclear antibody; CENP-B: centromere protein B; CRP: C-reactive protein; dcSSc: diffuse cutaneous SSc; ESR: erythrocyte sedimentation rate; ET-1: endothelin-1; lcSSc: limited cutaneous SSc; LVEF: left ventricular ejection fraction; NYHA: New York Heart Association; PASP: pulmonary artery systolic pressure; PDE5: phosphodiesterase-5; SSc: systemic sclerosis; TAPSE: tricuspid annular plane systolic excursion.