Table 1 Baseline characteristics according to survival endpoint
From: Proteomic biomarkers for survival in systemic sclerosis-associated pulmonary hypertension
Long surviving patients n = 19 | Short surviving patients n = 13 | |
|---|---|---|
Demographic characteristics | ||
Age, median (IQR) years | 60 (48.5–64) | 51 (46.6–69.2) |
Male gender, n (%) | 4 (21) | 5 (38.5) |
BMI, kg/m2, median (IQR) | 26.6 (23.8–31) | 23.9 (22.1–27.1) |
SSc cutaneous subtype, n (%) | ||
Limited | 5 (27.7) | 6 (50) |
Diffuse | 13 (72.2) | 6 (50) |
Autoantibodies, n (%) | ||
Anti-Scl-70 | 7 (36.8) | 0 (0) |
Anticentromere | 0 (0) | 2 (15.4) |
Other/unknown | 12 (63.2) | 11 (84.6) |
6-min walk test, median (IQR) | ||
Distance, meters | 337 (298–416) | 299 (225–476) |
Borg dyspnea score | 4 (3–5) | 4 (1–5) |
Biology, median (IQR) a | ||
BNP (pg/mL) n = 5 | 64 (30–134) | 57 (42–82.5) |
Urate (µmol/L) n = 5 | 273 (208–328) | 208 (207–227) |
WHO functional class, n (%) | ||
1–2 | 7 (36.8) | 1 (7.7) |
3 | 10 (52.6) | 10 (76.9) |
4 | 2 (10.5) | 2 (15.4) |
Long term-term supplemental O2, n (%) | 6 (31.6) | 9 (69.2) |
Comorbidities, n (%) | ||
Arterial hypertension | 6 (31.6) | 6 (46.1) |
Diabetes | 1 (5.3) | 2 (15.4) |
Coronary disease | 0 (0) | 0 (0) |
Chronic kidney disease | 0 (0) | 0 (0) |
Obesity | 3 (15.8) | 0 (0) |
Hemodynamics, median (IQR) | ||
RAP, mmHg | 7 (5.5–10) | 6 (4–9) |
Mean PAP, mmHg | 30 (28.5–39.5) | 38 (33–46) |
Cardiac Index, liter/minute/m2 | 3 (2.7–3.5) | 2,4 (2–2.8) |
PVR, Wood units | 4.4 (3–5.2) | 6.2 (4.2–10.7) |
PAWP, mmHg | 9.5 (7–12) | 12 (9–14) |
Pulmonary function tests, median (IQR) | ||
FVC, % of predicted | 62 (58–83.5) | 62.5 (53–81) |
FEV1, % of predicted | 64 (51–76) | 69.5 (57–75.5) |
TLC, % of predicted | 67 (59.76.5) | 68 (51.7–78) |
DLCO, % predicted | 39 (35–44.2) | 25 (21.7–30.5) |
DLCO/VA, % predicted | 63 (49.2–77) | 44.5 (39.5–51.7) |
PaO2 (mmHg) room air | 81 (67–83.3) | 54 (51–70.5) |
PaCO2 (mmHg) room air | 38 (34.9–42) | 37 (34.5–41) |
Radiological ILD pattern, n (%)a | ||
Nonspecific interstitial pneumonia | 5 (45) | 6 (50) |
Usual interstitial pneumonia | 6 (55) | 2 (16.7) |
Undetermined pattern | 0 (0) | 4 (33.3) |
Extent of ILD on CT, n (%)a | ||
Limited | 3 (25) | 3 (25) |
Extensive | 9 (75) | 9 (75) |
%Extent of ILD on CT, median (IQR) a | 52.5 (28.7–70) | 47.5 (27.5–66.2) |
First-line PH treatment, n (%) | ||
ERA | 17 (89.5) | 10 (76.9) |
PDE5i | 1 (5.3) | 2 (15.4) |
ERA + PDE5i | 1 (5.3) | 0 (0) |
Prostacyclin analogs + ERA + PDE5i | 0 (0) | 1 (8.7) |
Sequential oral combination PH therapy during follow-up, n (%) | 3 (15.8) | 4 (30.8) |
Other treatments, n (%) | ||
Diuretics | 8 (42.1) | 7 (54.8) |
Oral anticoagulation | 6 (31.6) | 4 (30.8) |
ILD treatment at PH diagnosis, n (%) | ||
None | 4 (21) | 7 (53.8) |
Glucocorticoids alone | 7 (36.8) | 3 (23.1) |
AZA | 1 (5.3) | 0 (0) |
MMF | 1 (5.3) | 1 (7.7) |
MMF + CYC | 1 (5.3) | 1 (7.7) |
Missing | 5 (26.3) | 1 (7.7) |
Duration of follow-up, median (IQR) days | 2967 (2412–3606) | 901 (571–997) |