Table 1 Baseline Characteristics and Lung Function of IPAH and MCTD-PH

Age, years

42.00 ± 14.16

44.28 ± 14.03

P = 0.362

BMI, kg/m2

23.79 ± 3.47

22.62 ± 3.40

P = 0.056

Sex, M/F

19/35

3/75

WHO functional class

Class I, I-II, II

23

26

Class II-III, III

25

49

Class III-IV, IV

3

6

NT-ProBNP, pg/ml

502.1 (77.35–1248)

512.3 (164.9–1678)

P = 0.333

FeNO, (ppb)

19 (14–27)

15 (8.75–21.5)

P = 0.003

PFT

FVC, L

2.93 ± 0.79

2.33 ± 0.64

P < 0.0001

FEV1, L

2.37 (1.94–2.76)

2.05 (1.50–2.34)

P < 0.0001

FEV1/FVC

82.18 ± 5.64

83.53 ± 6.99

P = 0.239

MEF50%

84.05 (67.83-104.35)

76.40 (61.48–96.90)

P = 0.122

MEF25%

62.45 (45.28–83.08)

60.55 (41.48–78.95)

P = 0.485

MMEF75/25%

68.17 ± 21.33

61.62 ± 21.28

P = 0.084

IOS

R5, cmH2O/(L/s)

3.79 (3.07–4.60)

4.32 (3.71–5.71)

P = 0.002

R5%

110.90 (93.75–127.80)

117.10 (101.75–153.20)

P = 0.052

R20, cmH2O/(L/s)

2.75 (2.45–3.54)

3.47 (2.63–4.39)

P = 0.001

R20%

95.70 (85.65-120.15)

110.80 (87.75-140.95)

P = 0.036