Background
Methods
Patients and diagnostic criteria
Data collection
Statistical analysis
Results
Characteristics of the patients
Variable | MCTD-PAH group (n = 46) | MCTD-non-PAH group (n = 73) | Statistical values | P | |
---|---|---|---|---|---|
Demographics | |||||
Sex [F/M, n (%)] | 44 (95.7)/2 (4.4) | 69 (94.5)/4 (5.5) | 0.000 | > 0.999 | |
Age at MCTD onset (years, \(\bar x\)±s) | 35.1 ± 12.8 | 36.5 ± 11.8 | -0.603 | 0.547 | |
Age at MCTD diagnosis (years, \(\overline{ x}\)±s) | 37.8 ± 13.4 | 39.8 ± 12.2 | -0.831 | 0.408 | |
Age at PAH onset (years, \(\bar x\)±s) | 38.9 ± 13.4 | ||||
Age at PAH diagnosis (years, \(\bar x\)±s) | 39.9 ± 13.7 | ||||
Height [m, M (P25-P75)] | 1.61 (1.58–1.64) | 1.61 (1.58–1.63) | -0.008 | 0.993 | |
Weight (kg, \(\bar x\)±s) | 60.76 ± 4.17 | 59.43 ± 3.50 | 1.874 | 0.063 | |
BMI (kg/m2, \(\bar x\)±s) | 23.49 ± 1.49 | 22.99 ± 1.32 | 1.875 | 0.064 | |
BSA (m2, \(\bar x\)±s) | 1.72 ± 0.06 | 1.71 ± 0.05 | 1.545 | 0.125 | |
Clinical manifestations [n(%)] | |||||
SLEDAI-2 K Activity | -0.052 | 0.958 | |||
Almost inactive | 20 | 31 | |||
Mild | 15 | 25 | |||
Moderate | 8 | 12 | |||
Severe | 3 | 5 | |||
SLEDAI-2 K scores | 5.00 (1.00-9.25) | 5.00 (4.00–9.00) | -0.907 | 0.364 | |
Raynaud’s phenomenon | 42 (91.3) | 67 (91.8) | 0.000 | > 0.999 | |
Sclerodactyly | 15 (32.6) | 17 (23.3) | 1.247 | 0.264 | |
Serositis | 29 (63.0) | 20 (27.4) | 14.803 | < 0.001 | |
Limbs edema | 8 (17.4) | 7 (9.6) | 1.559 | 0.212 | |
Fatigue | 26 (56.5) | 36 (49.3) | 0.587 | 0.443 | |
Arthralgia | 31 (67.4) | 43 (58.9) | 0.864 | 0.353 | |
Chest tightness | 35 (76.1) | 24 (32.9) | 21.076 | < 0.001 | |
Laboratory profiles | |||||
WBC (×109/L, \(\bar x\)±s) | 5.9 ± 2.8 | 7.6 ± 8.0 | -1.426 | 0.156 | |
RBC [×1012/L, M (P25-P75)] | 4.3(3.8–4.8) | 4.3(3.8–4.5) | -0.669 | 0.504 | |
PLT (×109/L, \(\bar x\)±s) | 185.0 ± 84.9 | 206.7 ± 101.8 | -1.207 | 0.230 | |
HGB (g/L, \(\bar x\)±s) | 124.6 ± 11.5 | 124.5 ± 12.1 | 0.079 | 0.937 | |
MCV (fL, \(\bar x\)±s) | 87.1 ± 3.3 | 86.4 ± 3.3 | 1.260 | 0.210 | |
RDW [%, \(\bar x\)±s] | 18.2 ± 3.4 | 13.8 ± 1.7 | 8.174 | < 0.001 | |
UA (µmol/L, \(\bar x\)±s) | 320.5 ± 132.1 | 270.8 ± 97.9 | 2.200 | 0.031 | |
CRP [mg/L, M (P25-P75)] | 4.2 (2.5–11.3) | 4.1 (2.5–8.9) | -0.612 | 0.541 | |
ESR [mm/hr, M (P25-P75)] | 29.5 (13.5–55.0) | 29.0 (12.0-43.5) | -0.972 | 0.331 | |
TSH [mU/L, M (P25-P75)] | 2.6 (1.1–4.9) | 1.3 (0.8–2.7) | -2.423 | 0.015 | |
FT3 (pmol/L, \(\bar x\)±s) | 3.0 ± 1.2 | 3.5 ± 1.0 | -3.130 | 0.002 | |
FT (pmol/L, \(\bar x\)±s) | 14.9 ± 3.3 | 15.4 ± 3.0 | -0.887 | 0.377 | |
C3 (g/L, \(\bar x\)±s) | 0.9 ± 0.3 | 1.0 ± 0.3 | -1.648 | 0.102 | |
C4 (g/L, \(\bar x\)±s) | 0.2 ± 0.1 | 0.4 ± 0.8 | -1.852 | 0.067 | |
IgA [g/L, M (P25-P75)] | 2.1 (1.5–3.3) | 2.7 (2.0-3.4) | -1.839 | 0.066 | |
IgG [g/L, M (P25-P75)] | 17.0(12.1–27.5) | 16.7 (13.1–20.0) | -0.854 | 0.393 | |
IgM (g/L, \(\bar x\)±s) | 1.3 ± 0.7 | 1.4 ± 0.9 | -0.534 | 0.594 | |
D-Dimer [mg/L, M (P25-P75)] | 0.5 (0.2–1.4) | 0.5 (0.3–0.8) | -1.561 | 0.118 | |
Fg [g/L, M (P25-P75)] | 2.6 (2.3–2.9) | 2.6 (2.3-3.0) | -0.014 | 0.989 | |
PT [s, M (P25-P75)] | 18.5 (17.5–19.5) | 18.9 (18.4–19.6) | -1.177 | 0.239 | |
APTT (s, \(\bar x\)±s) | 27.8 ± 4.9 | 28.3 ± 5.0 | -0.526 | 0.600 | |
Autoantibody profiles [positive number, n(%)] | |||||
Nuclear speckled | 38 (82.6) | 54 (74.0) | 1.200 | 0.273 | |
Nuclear homogeneous | 1 (2.2) | 1 (1.4) | — | 1.00 | |
Nuclear membrane | 1 (2.2) | 1 (1.4) | — | 1.00 | |
Nucleolar | 1 (2.2) | 1 (1.4) | — | 1.00 | |
Nuclear centromeric | 1 (2.2) | 4 (5.5) | 0.165 | 0.685 | |
Cytoplasmic | 7 (15.2) | 12 (16.4) | 0.031 | 0.859 | |
U1-RNP | 35 (76.1) | 48 (65.8) | 1.428 | 0.232 | |
Sm | 3 (6.5) | 6 (8.2) | — | 1.00 | |
SS-A | 14 (30.4) | 13 (17.8) | 2.565 | 0.109 | |
RO52 | 10 (21.7) | 12 (16.4) | 0.526 | 0.468 | |
SS-B | 5 (10.9) | 3 (4.1) | 1.120 | 0.290 | |
SCL70 | 1 (2.2) | 1 (1.4) | — | 1.00 | |
Jo-1 | 1 (2.2) | 2 (2.7) | — | 1.00 | |
dsDNA | 1 (2.2) | 1 (1.4) | — | 1.00 |
Laboratory findings
Echocardiographic findings
Variable | MCTD-PAH group (n = 46) | MCTD-non-PAH group (n = 73) | Statistical values | P |
---|---|---|---|---|
IVSTd [cm, M (P25-P75)] | 0.8 (0.8–0.9) | 0.8 (0.8–0.9) | -1.373 | 0.170 |
LVPWTd [cm, M (P25-P75)] | 0.8 (0.8–0.9) | 0.80 (0.8–0.9) | -0.969 | 0.333 |
LVEF [%, M (P25-P75)] | 59.0 (58.0-60.3) | 61.0 (60.0-61.5) | -2.970 | 0.003 |
LVDd (cm, \(\bar x\)±s) | 4.5 ± 0.5 | 4.7 ± 0.4 | -3.130 | 0.003 |
AoD [cm, M (P25-P75)] | 2.8 (2.8–3.1) | 3.0 (2.7–3.3) | -0.757 | 0.449 |
PA (m/s, \(\bar x\)±s) | 0.8 ± 0.1 | 0.9 ± 0.2 | -2.317 | 0.022 |
LVDs [cm, M (P25-P75)] | 3.1 (2.8–3.3) | 3.2 (3.0-3.3) | -1.737 | 0.082 |
LAD [cm, M (P25-P75)] | 3.5 ± 0.7 | 4.4 ± 0.6 | -2.079 | 0.038 |
E/A [M (P25-P75)] | 0.9 (0.8–1.4) | 1.2 (0.8–1.4) | -0.764 | 0.445 |
Pericardial effusion [P/N, n (%)] | 17 (37.0)/29 (63.0) | 15 (32.6)/58 (79.5) | 3.865 | 0.049 |
PASP [mmHg, M (P25-P75)] | 67.0 (48.5–90.0) | < 36 | — | |
BNP [pg/mL, M (P25-P75)] | 180.0 (63.8-478.5) | 57.3 (22.8–112.0) | -5.621 | < 0.001 |
WHO Fc [I-II/III-IV, n (%)] | 25 (54.4)/21 (45.7) | 72 (98.6)/1 (1.4) | 36.719 | < 0.001 |
Factors associated with PAH
Univariable binary logistic regression analysis | Multivariable binary logistic regression analysis | |||||||
---|---|---|---|---|---|---|---|---|
Variable | P | OR | 95% CI | P | OR | 95% CI | ||
Lower limit | Upper limit | Lower limit | Upper limit | |||||
RDW | < 0.001 | 2.199 | 1.663 | 2.906 | < 0.001 | 2.128 | 1.497 | 3.026 |
BNP | < 0.001 | 1.005 | 1.002 | 1.009 | 0.324 | 1.002 | 0.998 | 1.005 |
UA | 0.024 | 1.004 | 1.001 | 1.007 | 0.791 | 1.001 | 0.995 | 1.006 |
TSH | 0.379 | 1.055 | 0.936 | 1.189 | ||||
FT3 | 0.009 | 0.624 | 0.437 | 0.891 | 0.280 | 0.737 | 0.424 | 1.282 |
LVEF | 0.445 | 0.980 | 0.932 | 1.031 | ||||
LVDd | 0.002 | 0.219 | 0.084 | 0.571 | 0.341 | 1.980 | 0.485 | 8.073 |
PA | 0.025 | 0.030 | 0.001 | 0.646 | 0.389 | 0.138 | 0.002 | 12.428 |
LAD | 0.142 | 0.608 | 0.313 | 1.182 | ||||
Pericardial effusion | 0.052 | 0.441 | 0.193 | 1.007 | 0.854 | 0.878 | 0.219 | 3.518 |
Chest tightness | < 0.001 | 0.154 | 0.067 | 0.355 | 0.095 | 0.169 | 0.021 | 1.366 |
Serositis | < 0.001 | 0.221 | 0.100 | 0.487 | 0.593 | 1.736 | 0.230 | 13.090 |