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Table 1 Patient baseline characteristic

From: Cross-cultural validation of the Chinese version of the EmPHasis-10 questionnaire in connective tissue disease patients with pulmonary arterial hypertension and its relationship with risk stratification

Characteristic

Values (N = 75)

Female, %

98.7

Age, years

36.4 ± 11.9

Time since initial PAH diagnosis, months

49.5 (39.3–69.7)

BMI, kg/m2

22.4 ± 3.4

Education

 Middle school, %

23.2

 High school, %

15.9

 College, %

60.9

Marital status

 Married, %

74.3

 Unmarried, %

23.1

 Divorce,%

2.6

Smoking

 Yes, %

1.9

 No, %

98.1

CTD etiology

 SLE, %

56.7

 SS, %

15.0

 SSc, %

11.8

 UCTD, %

8.3

 RA, %

1.7

Clinical features

 Arthritis, %

30.7

 ILD, %

10.7

WHO functional class

 I, %

14.9

 II, %

60.9

 III, %

24.3

 NT-proBNP, pg/ml

95.0 (39.0–390.0)

Echocardiography

 PASP, mmHg

55.9 ± 24.5

 TAPSE, mm

18.9 ± 3.7

 RV diameter, mm

40.9 ± 6.5

 RV internal dimension, mm

24.6 ± 6.7

 Pericardial effusion, %

17.7

 LVEF, %

68.3 ± 6.9

RHC

 mRAP, mmHg

8.5 ± 2.9

 mPAP, mmHg

38.9 ± 13.7

 PAWP, mmHg

11.9 ± 3.6

 PVR, WU

4.8 ± 3.1

 CI, L/(min × m2)

3.7 ± 0.8

Treatment

Glucocoticoid

 High-dose (≥ 40 mg/day), %

22.7

 Medium-dose (15 mg/day < x < 40 mg/day), %

13.3

 Low-dose (≤ 15 mg/day), %

64

Immunosuppressant

 CYC, %

30.5

 MMF, %

30.5

 TAC, %

40.7

 HCQ, %

69.5

 ≥ 2, %

78.0

PAH medication

 ERA, %

54.2

 PDE5-I, %

55.9

 PG, %

3.4

 ≥ 2, %

16.3

Diuretic, %

66.1

Digoxin, %

37.3

  1. Date expressed as mean ± standard deviation or median (Interquartile range:25%-75%)
  2. WHO, World Health Organization; SLE, systemic lupus erythematosus; SS, sjogren syndrome; SSc, systemic sclerosis; UCTD, undifferentiated connective tissue diseases; RA, rheumatoid arthritis; ILD, Interstitial lung disease; NT-proBNP, N-terminal pro-brain natriuretic peptide; PASP, pulmonary arterial systolic pressure; TAPSE, tricuspid annular plane systolic excusion; RV, right ventricular; LVEF, left ventricular ejection fraction; RHC, right heart catheterization; mRAP, mean right atrial pressure; mPAP, mean pulmonary arterial pressure; PAWP, pulmonary arterial wedge pressure; PVR, pulmonary vascular resistance; CI, cardiac index; CYC, cyclophosphamide; MMF, mycophenolate mofetil; TAC. tacrolimus; HCQ, hydroxychloroquine; ERA, endothelin receptor antagonist; PDE5-I, phosphodiesterase inhibitor; PG, prostacyclin analogue