Table 1 Characteristics and differences of the 3 clusters

Variables

Age, years

40.39(31.06–53.66)

39.44(31.24–53.81)

46.19(33.64–59.13)

Female, n (%)

86(93.5)

62(98.4)

27(93.1)

CTD characteristics

Course of CTD, months

(Time from diagnosis of CTD to diagnosis of PAH)

12.53(0.03–83.43)

0.50(0.03–125.20)

52.50(0.10-123.75)

Protopathic CTDs

SLE, n (%)

34(37)

40(63.5) **

10(34.5) ^##

pSS, n (%)

27(29.3)

20(31.7)

7(24.1)

SSc, n (%)

11(12.0)

1(1.6) *

5(17.2) ^##

MCTD, n (%)

5(5.4)

2(3.2)

4(13.8)

UCTD, n (%)

10(10.9)

0(0) **

2(6.9) ^#

Others, n (%)

5(5.4)

0(0)

1(3.4)

NLR

2.06(1.57–2.90) ^$$$

2.52(1.48–3.75)

8.00(5.29–10.97) ^###

Monocyte, 10^9/L

0.46(0.34–0.62)

0.32(0.20–0.53) **

0.45(0.32–0.70)

eGFR, mL/min/1.73 m^2

104.54 ± 29.78

116.89 ± 42.04

100.41 ± 43.73

ESR, mm/h

10.50(4.25-22.00) ^$$$

52.00(26.00–86.00) ***

53.00(39.00–99.00)

CRP, mg/L

3.22(2.53–4.53) ^$$$

5.14(2.47-9.00) *

24.70(10.45–64.25) ^###

IgG, g/L

11.70(9.85–15.41) ^$

22.30(15.00-33.10) ***

17.50(10.58–23.05) ^##

C3, g/L

0.91 ± 0.19 ^$$$

0.59 ± 0.22 ***

0.73 ± 0.22 ^##

C4, g/L

0.19 ± 0.06

0.11 ± 0.04 ***

0.17 ± 0.09 ^###

Anti-Ro52+, n (%)

28(35.9)

38(64.4) ***

11(50)

Anti-Ro60+, n (%)

37(48.7)

43(78.2) ***

11(50) ^#

Anti-U1-snRNP+, n (%)

19(24.7) ^$$

31(52.5) ***

13(61.9)

Anti-dsDNA+, n (%)

11(15.1)

18(30.0) *

7(31.8)

Anti-Sm+, n (%)

8(10.4)

17(28.8) **

5(23.8)

Anti-centromere+, n (%)

12(15.6)

6(10.2)

3(14.3)

PAH characteristics

Course of PAH, months (Symptom-to-diagnosis time for PAH)

14.52(3.44–40.26)

6.10(1.57–25.40)

4.37(1.49–33.54)

6MWD, m

407.45 ± 128.10

436.33 ± 109.97

401.80 ± 145.24

WHO-FC(I/II/III/IV), n

4/42/44/2

1/36/24/1

1/11/15/2

NT-pro BNP, pg/mL

844.10(231.00-2923.00)

480.75(179.93-1181.75)

1449.00(370.50–2782.00)

2018WSPH risk stratification

Low/intermediate/ high risk, n

33/34/25

24/30/8

6/18/5

Right cardiac catheterization parameters

Heart rate, beats/min

83.00(75.25-92.00)

81.00(76.00-95.75)

90.00(82.00-100.00)

mPAP, mmHg

44.00(37.00-51.75)

37.00(29.00–49.00) **

39.00(32.50–52.50)

PVR, Wood

8.78(6.02–13.55) ^$

5.36(3.86–9.16) ***

7.20(4.60–9.57)

RAP, mmHg

5.00(3.00–9.00)

5.00(3.00–7.00)

6.00(2.50-8.00)

CI, L/min/m²

2.70(2.03–3.11)

3.20(2.75–3.90) ***

2.79(2.10–3.32) ^#

Two-dimensional echocardiography parameters

RADI, mm/m^2

27.30 ± 4.61

26.40 ± 4.65

25.08 ± 4.66

RVDDI, mm/m^2

27.69 ± 4.40

26.79 ± 4.54

25.79 ± 5.21

TAPSE/PASP, mm/mmHg

0.21(0.16–0.28) ^$

0.27(0.18–0.34) *

0.30(0.19–0.36)

Pericardial effusion, n (%)

33(35.9)

28(45.2)

13(44.8)