Introduction
Methods
Study design
Current analysis population
Data collection and analysis
Results
Baseline characteristics of patients with normal mPAP, BoPAP and PAH
Patient groups (N =244) | P-value | ||||
---|---|---|---|---|---|
Variable | Normal mPAP (N =148) | BoPAP (N =36) | PAH (N =60) | BoPAP versus normal mPAP | PAH versus BoPAP |
Demographic and clinical parameters
| |||||
Age, years, Na | 148 | 36 | 59 | ||
Mean ± SD | 54.3 ± 12.0 | 58.4 ± 9.5 | 61.6 ± 9.7 | ||
Median, Q1 to Q3 | 55.0, 46.0 to 63.5 | 58.5, 52.5 to 63.5 | 62.0, 56.0 to 68.0 | ||
Minimum, maximum | 26.0, 78.0 | 43.0, 80.0 | 33.0, 80.0 | ||
Gender, n | 148 | 36 | 60 | ||
Male, n (%) | 18 (12.2) | 6 (16.7) | 12 (20.0) | ||
Female, n (%) | 130 (87.8) | 30 (83.3) | 48 (80.0) | ||
WHO FC, N | 139 | 34 | 60 | <0.05 | |
I/II, n (%) | 127 (91.4) | 28 (82.4) | 37 (61.7) | ||
III/IV, n (%) | 12 (8.6) | 6 (17.6) | 23 (38.3) | ||
6-minute walk distance, N | 108 | 27 | 48 | ||
Mean ± SD | 422.4 ± 110.4 | 397.4 ± 102.3 | 391.4 ± 109.8 | ||
Median, Q1 to Q3 | 432.5, 364.5 to 502.0 | 414.5, 341.0 to 472.0 | 395.0, 318.0 to 460.5 | ||
SSc disease duration (years), N | 147 | 36 | 60 | ||
Mean ± SD | 11.0 ± 8.0 | 10.0 ± 5.0 | 14.0 ± 12.0 | ||
Median, Q1 to Q3 | 8.0, 5.0 to 13.0 | 9.0, 6.0 to 15.0 | 11.0, 6.0 to 16.0 | ||
SSc subtype, N | 146 | 36 | 59 | ||
Limited, n (%) | 90 (61.6) | 20 (55.6) | 44 (74.6) | ||
Diffuse, n (%) | 45 (30.8) | 13 (36.1) | 10 (16.9) | ||
Overlap/mixed CTD, n (%) | 11 (7.5) | 3 (8.3) | 5 (8.5) | ||
Presence of peripheral edema, n/N (%) | 8/147 (5.4) | 7/36 (19.4) | 11/60 (18.3) | <0.01 | |
Presence of telangiectasias, n/N (%) | 108/148 (73.0) | 22/36 (61.1) | 52/60 (86.7) | <0.005 | |
FVC % predicted, N | 148 | 36 | 60 | ||
Mean ± SD | 92.5 ± 17.6 | 88.7 ± 14.5 | 92.0 ± 19.7 | ||
Median, Q1-Q3 | 91.9, 79.9 to 102.7 | 87.1, 80.0 to 96.5 | 89.5, 77.2 to 102.5 | ||
FVC % predicted /DLCO % predicted, N | 148 | 36 | 60 | <0.01 | |
Mean ± SD | 1.9 ± 0.4 | 1.9 ± 0.4 | 2.3 ± 0.7 | ||
Median, Q1 to Q3 | 1.8, 1.5 to 2.1 | 2.0, 1.5 to 2.2 | 2.2, 1.8 to 2.5 | ||
TLC % predicted, N | 127 | 28 | 54 | <0.05 | |
Mean ± SD | 92.8 ± 21.9 | 84.8 ± 14.4 | 88.1 ± 21.6 | ||
Median, Q1 to Q3 | 92.5, 80.0 to 106.0 | 83.0, 74.0 to 95.0 | 87.3, 78.0 to 100.3 | ||
Serum laboratory tests
| |||||
Presence of anti-centromere antibodies | <0.05 | ||||
n/N (%) | 49/141 (34.8) | 10/34 (29.4) | 30/56 (53.6) | ||
Log10 NT-proBNP (pg/ml), N | 141 | 34 | 56 | <0.05 | |
Mean ± SD | 2.0 ± 0.5 | 2.2 ± 0.5 | 2.4 ± 0.5 | ||
Median, Q1 to Q3 | 2.1, 1.7 to 2.3 | 2.2, 1.9 to 2.5 | 2.3, 2.0 to 2.8 | ||
Serum urate, mg/100 ml, N | 141 | 34 | 56 | <0.01 | |
Mean ± SD | 4.7 ± 1.4 | 4.7 ± 1.3 | 5.6 ± 1.4 | ||
Median, Q1 to Q3 | 4.4, 3.7 to 5.3 | 4.5, 3.7 to 5.4 | 5.6, 4.6 to 6.5 | ||
Log10 estimated GFR | 141 | 34 | 55 | ||
1.9 ± 0.1 | 1.9 ± 0.1 | 1.9 ± 0.1 | |||
1.9, 1.9 to 2.0 | 1.9, 1.8 to 2.0 | 1.9, 1.8 to 2.0 | |||
Echocardiography
| |||||
Left atrium diameter, mm, N | 148 | 36 | 60 | <0.001 | |
Mean ± SD | 30.2 ± 5.1 | 33.9 ± 3.6 | 31.8 ± 5.4 | ||
Median, Q1 to Q3 | 31.0, 27.8 to 34.0 | 33.5, 31.0 to 37.0 | 32.0, 30.0 to 36.0 | ||
Right atrium area, cm2, N | 142 | 33 | 58 | <0.05 | |
Mean ± SD | 12.8 ± 4.7 | 14.7 ± 5.9 | 16.8 ± 5.8 | ||
Median, Q1 to Q3 | 12.0, 10.0 to 14.3 | 12.4, 10.5 to 18.0 | 15.8, 13.4 to 20.0 | ||
TAPSE (mm), N | 138 | 33 | 53 | ||
Mean ± SD | 23.2 ± 4.3 | 23.8 ± 5.1 | 21.1 ± 4.3 | ||
Median, Q1 to Q3 | 23.7, 20.4 to 26.0 | 23.0, 20.0 to 27.0 | 20.8, 19.0 to 23.0 | ||
TR velocity (m/s), N | 140 | 35 | 58 | <0.001 | <0.05 |
Mean ± SD | 2.3 ± 0.4 | 2.7 ± 0.3 | 3.0 ± 0.7 | ||
Median, Q1 to Q3 | 2.3, 2.2 to 2.6 | 2.7, 2.5 to 2.9 | 2.9, 2.5 to 3.5 | ||
Electrocardiography
| |||||
Presence of right axis deviation, n/N (%) | 2/138 (1.4) | 1/30 (3.3) | 8/58 (13.8) | ||
Right heart catheterization
| |||||
PAWP, mmHg, N | 148 | 36 | 60 | <0.001 | |
Mean ± SD | 7.7 ± 3.3 | 10.8 ± 3.0 | 9.9 ± 3.3 | ||
Median, Q1 to Q3 | 8.0, 5.0 to 10.0 | 11.0, 9.0 to 13.0 | 10.5, 7.5 to 12.5 | ||
TPG, mmHg, N | 148 | 36 | 60 | <0.001 | <0.001 |
Mean ± SD | 7.9 ± 2.7 | 11.6 ± 2.8 | 22.3 ± 9.7 | ||
Median, Q1 to Q3 | 7.0, 6.0 to 10.0 | 11.0, 10.0 to 13.0 | 20.0, 15.0 to 28.0 | ||
DWG, mmHg, N | 148 | 36 | 60 | <0.001 | |
Mean ± SD | 1.8 ± 2.7 | 3.0 ± 5.4 | 10.7 ± 8.1 | ||
Median, Q1 to Q3 | 1.0, 0.0 to 3.0 | 2.0, 0.5 to 5.0 | 8.0, 5.0 to 17.0 | ||
PVR, dyn.sec/cm5, N | 148 | 36 | 60 | <0.001 | <0.001 |
Mean ± SD | 128.0 ± 50.8 | 179.8 ± 58.7 | 375.8 ± 217.0 | ||
Median, Q1 to Q3 | 121.5, 91.3 to 153.9 | 170.3, 147.8 to 187.8 | 295.5, 236.0 to 419.1 |
Univariable logistic regression analysis
BoPAP versus normal PAP
Variable | Odds ratios (95% confidence interval) | P-value | ||
---|---|---|---|---|
BoPAP versus normal mPAP | PAH versus BoPAP | BoPAP versus normal mPAP | PAH versus BoPAP | |
Demographic and clinical parameters
| ||||
Age, years | 1.03 (1.00, 1.07) | 1.04 (0.99, 1.08) | ||
Gender, female versus male | 0.69 (0.25, 1.89) | 0.80 (0.27, 2.36) | ||
WHO FC, III/IV versus I/II | 2.27 (0.78, 6.56) | 2.90 (1.04, 8.08) | <0.05 | |
6-minute walk distance | 1.00 (0.99, 1.00) | 1.00 (1.00, 1.00) | ||
SSc disease duration, years | 0.99 (0.94, 1.04) | 1.05 (1.00, 1.11) | ||
SSc disease subtype | <0.05 | |||
dcSSc versus lcSSc | 1.30 (0.59, 2.85) | 0.35 (0.13, 0.93) | ||
Overlap/mixed CTD versus lcSSc | 1.23 (0.31, 4.81) | 0.76 (0.17, 3.48) | ||
Presence of telangiectasias | 0.58 (0.27, 1.25) | 4.13 (1.52, 11.26) | <0.01 | |
Presence of peripheral edema | 4.19 (1.41, 12.46) | 0.93 (0.32, 2.67) | <0.05 | |
FVC predicted | 0.99 (0.97, 1.01) | 1.01 (0.99, 1.04) | ||
FVC % predicted /DLCO % predicted | 1.24 (0.54, 2.82) | 3.41 (1.36, 8.54) | <0.01 | |
TLC % predicted | 0.98 (0.96, 1.00) | 1.01 (0.99, 1.04) | ||
Serum laboratory
| ||||
Presence of anti-centromere antibodies | 0.78 (0.35, 1.77) | 2.77 (1.12, 6.85) | <0.05 | |
Log10 NT-proBNP, pg/ml | 2.65 (1.17, 5.98) | 1.69 (0.71, 4.05) | <0.05 | |
Serum urate, mg/100 ml | 1.01 (0.76, 1.33) | 1.73 (1.20, 2.48) | <0.01 | |
Log10 estimated GFR | 0.54 (0.03, 9.44) | 0.04 (0.00, 1.77) | ||
Echocardiography
| ||||
Left atrium diameter, mm | 1.22 (1.10, 1.35) | 0.89 (0.80, 1.00) | <0.001 | <0.05 |
Right atrium area, cm2 | 1.07 (1.00, 1.14) | 1.07 (0.99, 1.17) | ||
TAPSE, mm | 1.03 (0.94, 1.12) | 0.88 (0.80, 0.98) | <0.05 | |
TR velocity, m/s | 25.10 (6.07, 103.74) | 3.15 (1.30, 7.67) | <0.0001 | <0.05 |
Electrocardiography
| ||||
Right axis deviation | 2.35 (0.21, 26.74) | 4.64 (0.55, 38.99) | ||
Right heart catheterization
| ||||
PAWP, mmHg | 1.39 (1.21, 1.61) | 0.91 (0.79, 1.04) | <0.0001 | |
TPG, mmHg | 1.57 (1.33, 1.85) | 1.72 (1.35, 2.19) | <0.0001 | <0.0001 |
DWG, mmHg | 1.10 (0.99, 1.21) | 1.27 (1.12, 1.44) | <0.0005 | |
PVR, dyn.sec/cm5 | 1.01 (1.01, 1.02) | 1.03 (1.01, 1.04) | <0.0001 | <0.0001 |