Data of patients with SSc according to the presence of pulmonary disease.
| Pulmonary Disease, n = 13 | No Pulmonary Disease, n = 16 | P | |
|---|---|---|---|
| Age, yrs | 50 (44–62) | 53 (42–63) | 0.82 |
| Women, n (%) | 13 (100) | 15 (93) | > 0.99 |
| Disease duration, yrs | 8 (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 | ||
| dcSSc | 7 (54) | 3 (18) | |
| lcSSc | 6 (46) | 13 (82) | |
| Drug therapies, n (%) | |||
| Mycophenolate mofetil | 8 (62) | 2 (13) | 0.02 |
| Methotrexate | 3 (23) | 4 (25) | > 0.99 |
| Calcium channel blockers | 8 (62) | 5 (31) | 0.14 |
| PDE5 inhibitors | 3 (23) | 0 | 0.08 |
| ET-1 receptor inhibitors | 2 (15) | 0 | 0.19 |
| Pulmonary involvement, n (%) | |||
| Pulmonary hypertension | 5 (38) | – | |
| Interstitial lung disease | 4 (31) | – | |
| Both | 4 (31) | – | |
| Raynaud phenomenon, n (%) | 10 (77) | 11 (69) | 0.70 |
| Modified Rodnan skin score | 17 (12–21) | 10 (7–14) | 0.02 |
| Valentini Disease Activity Index | 3 (1–3.5) | 0.5 (0–1.2) | 0.002 |
| Medsger Disease Severity Scale | 6 (6–8) | 2 (1–3) | < 0.001 |
| NYHA functional class, n (%) | 0.03 | ||
| Class I | 9 (69) | 16 (100) | |
| Class ≥ II | 4 (31) | 0 | |
| Autoantibodies, n (%) | |||
| ANA+ | 13 (100) | 16 (100) | > 0.99 |
| CENP-B+ | 5 (38) | 11 (68) | 0.14 |
| Scl-70+ | 3 (23) | 0 | 0.08 |
| CRP, mg/L | 3.1 (1.2–6.8) | 2.0 (0.9–3.4) | 0.33 |
| ESR, mm/h | 30 (18–38) | 10 (4–16) | 0.02 |
| 25(OH)D, n (%) | 0.21 | ||
| Normal (≥ 30 ng/mL) | 0 | 2 (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, mmHg | 35 (3–68) | 29 (25–36) | 0.02 |
| TAPSE, mm | 22 (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.