Characteristics | All SSc | SSc without PAH | SSc with PAH | p |
---|---|---|---|---|
Patients, n | 135 | 115 | 20 | |
Sex (M/F) | 11/124 | 10/105 | 1/19 | 1.000 |
SSc limited/diffuse | 96/39 | 80/33 | 15/5 | 0.794 |
Age, yrs, at onset, median (IQR) | 52 (43–59) | 51 (42–58) | 53.5 (48–65.5) | 0.192 |
Age, yrs, enrollment, median (IQR) | 61 (55–59) | 60 (53–68) | 67.5 (62–74) | 0.019 |
Disease duration, yrs, at enrollment, median (IQR) | 6 (2–15) | 6 (3–15) | 6 (3–15.5) | 0.571 |
ACA + (%) | 28 (49) | 57 (49) | 10 (50) | 1.000 |
Anti-Scl70 + (%) | 28 (21) | 24 (21) | 4 (20) | 1.000 |
Skin score, median (IQR) | 5.5 (4–12) | 5 (4–12) | 6 (2–18) | 0.664 |
Acral ulcers (%) | 33 (24) | 30 (26) | 3 (15) | 0.402 |
Calcinosis (%) | 23 (17) | 18 (16) | 5 (25) | 0.335 |
Esophageal dysmotility (%) | 42 (31) | 36 (31) | 6 (30) | 1.000 |
ILD (%) | 55 (41) | 44 (38) | 11 (55) | 0.221 |
Kazerooni score in ILD patients, median (IQR) | 4 (1–7) | 3.5 (1–7.25) | 4 (1–6) | 0.726 |
BNP, pg/ml, median (IQR) | 30 (18–61) | 30 (18–49) | 74.5 (29–196.5) | < 0.001 |
NT-proBNP, pg/ml, median (IQR) | 86 (40–188) | 84 (39–181) | 189 (44–665) | 0.061 |
Creatininemia, mg/dl median (IQR) | 0.9 (0.8–1) | 0.89 (0.8–1) | 1.15 (0.9–1.3) | 0.003 |
DLCO theoretical %, median (IQR) | 75 (63–84) | 77 (66–85) | 58 (50–64) | < 0.001 |
FVC theoretical %, median (IQR) | 101 (87–117) | 102 (89–118) | 86 (75–109) | 0.025 |
IQR: interquartile range; ILD: interstitial lung disease; ACA: anticentromere antibodies; SSc: systemic sclerosis; PAH: pulmonary arterial hypertension; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-brain natriuretic peptide; DLCO: diffusion capacity for carbon monoxide; FVC: forced vital capacity.