Table 3 Clinical characteristics at the baseline

Sex (male), n (%)

30 (13.1)

12 (15.2)

4 (15.4)

14 (11.3)

Age (year), median (IQR)

62.0 (51.0–70.0)

64.0 (51.0–70.0)

61.5 (54.0–72.0)

62.0 (49.0–69.5)

Height (cm), median (IQR)

 - N missing

154.6 (149.2–160.0)

20 (8.7)

155.2 (149.1–161.5)

3 (3.8)

154.4 (151.0–162.8)

1 (3.8)

153.0 (149.0–158.1)

16 (12.9)

Body weight (kg), median (IQR)

 - N missing

50.0 (44.0–58.9)

20 (8.7)

50.2 (44.5–56.0)

3 (3.8)

51.0 (47.9–57.0)

1 (3.8)

49.0 (41.7–59.9)

16 (12.9)

Diagnosis, n (%)

- Systemic sclerosis

186 (81.2)

70 (88.6)

19 (73.1)

97 (78.2)

- Mixed connective tissue disease

22 (9.6)

6 (7.6)

5 (19.2)

11 (8.9)

- Systemic lupus erythematosus

17 (7.4)

1 (1.3)

1 (3.8)

15 (12.1)

- Polymyositis/Dermatomyositis

8 (3.5)

2 (2.5)

2 (7.7)

4 (3.2)

- Primary Sjögren’s syndrome

7 (3.1)

0 (0.0)

0 (0.0)

7 (5.6)

Classification of PH, n (%)

- Group 1, PAH

143 (62.4)

-

22 (84.6)

121 (97.6)

- Group 2, PH associated with

left heart disease

15 (6.6)

-

4 (15.4)

11 (8.9)

- Group 3, PH associated with

lung diseases and/or hypoxia

50 (21.8)

-

11 (42.3)

39 (31.5)

- Group 4, PH associated with

chronic pulmonary artery obstruction

5 (2.2)

-

1 (3.8)

4 (3.2)

- Group 5, PH with unclear and/or

multifactorial mechanisms

0 (0)

-

0 (0)

0 (0)

History of smoke, n (%)

83 (36.2)

37 (46.8)

11 (42.3)

35 (28.2)

Comorbidities, n (%)

- Interstitial lung disease

104 (45.4)

40 (50.6)

19 (73.1)

45 (36.3)

- Chronic obstructive pulmonary disease

3 (1.3)

2 (2.5)

0 (0)

1 (0.8)

- Bronchial asthma

4 (1.7)

1 (1.3)

0 (0)

3 (2.4)

- Cardiac diseases

19 (8.3)

5 (6.3)

3 (11.5)

11 (8.9)

- Hypertension

48 (21.0)

17 (21.5)

6 (23.1)

25 (20.2)

- Chronic kidney disease

4 (1.7)

0 (0)

0 (0)

4 (3.2)

- Hepatic diseases

12 (5.2)

5 (6.3)

3 (11.5)

4 (3.2)

- Rheumatoid arthritis

18 (7.9)

7 (8.9)

2 (7.7)

9 (7.3)

- Secondary Sjögren’s syndrome

45 (19.7)

20 (25.3)

3 (11.5)

22 (17.7)

- Malignancy

3 (1.3)

2 (2.5)

0 (0)

1 (0.8)

mRSS, median (IQR)

 - N missing

6.0 (2.0–12.0)

104 (45.4)

10.0 (2.0–21.0)

35 (44.3)

7.0 (0.0–10.0)

8 (30.8)

4.0 (2.0–8.0)

61 (49.2)

Skin ulcer (presence), n (%)

 - N missing

50 (25.4)

32 (14.0)

22 (29.3)

4 (5.1)

5 (20.8)

2 (7.7)

23 (23.5)

26 (21.0)

WHO functional class, n (%)

- I

- II

- III

- IV

 - N missing

26 (11.4)

61 (26.6)

46 (20.1)

14 (6.1)

82 (35.8)

18 (22.8)

17 (21.5)

8 (10.1)

1 (1.3)

35 (44.3)

3 (11.5)

8 (30.8)

5 (19.2)

0 (0)

10 (38.5)

5 (4.0)

36 (29.0)

33 (26.6)

13 (10.5)

37 (29.8)

emPHasis-10, median (IQR)

 - N missing

10.5 (2.5–27.5)

225 (98.2)

10.0 (2.0–18.0)

77 (97.5)

-

26 (100)

20.0 (3.0–37.0)

122 (98.4)

6-minute walk test

- Distance (m), median (IQR)

 - N missing

- SpO₂ (%), median (IQR)

 - N missing

353.5 (251.3–430.0)

137 (59.8)

90.0 (85.0–95.0)

139 (60.7)

380.0 (270.0–470.0)

46 (58.2)

95.0 (90.0–97.0)

46 (58.2)

395.0 (240.0–430.0)

13 (50.0)

90.0 (87.0–95.0)

13 (50.0)

334.5 (245.0–395.0)

78 (62.9)

86.0 (82.0–93.0)

80 (64.5)

Blood test

- Hemoglobin (g/dL)

 - N missing

12.3 (11.1–13.5)

13 (5.7)

12.3 (11.0–13.2)

3 (3.8)

12.2 (11.5–13.2)

0 (0)

12.4 (11.1–13.9)

10 (8.1)

- Uric acid (mg/dL)

 - N missing

5.1 (4.1–6.4)

22 (9.6)

4.9 (3.9–5.6)

4 (5.1)

4.5 (4.1–6.6)

1 (3.8)

5.3 (4.3–7.0)

17 (13.7)

- Haptoglobin (mg/dL)

 - N missing

101.0 (67.0–155.0)

199 (86.9)

108.0 (81.0–155.0)

58 (73.4)

107.0 (67.0–147.0)

24 (92.3)

52.0 (34.0–163.0)

117 (94.4)

- BNP (pg/mL)

 - N missing

58.2 (25.1–220.3)

87 (38.0)

43.8 (15.2–80.8)

32 (40.5)

36.8 (16.5–49.5)

9 (34.6)

135.4 (39.5–427.2)

46 (37.1)

- NT-proBNP (pg/mL)

 - N missing

200.0 (101.0–893.0)

134 (58.5)

121.0 (67.1–190.0)

38 (48.1)

198.0 (65.0–780.2)

13 (50.0)

810.0 (298.0–4385.0)

83 (66.9)

- KL-6 (U/mL)

 - N missing

524.0 (326.0–985.0)

96 (41.9)

440.0 (256.0–1338.0)

26 (32.9)

669.0 (453.0–1576.0)

9 (34.6)

518.0 (340.0–910.0)

61 (49.2)

Transthoracic echocardiography

- LVDd (mm)

 - N missing

42.0 (38.0–45.3)

66 (28.8)

42.5 (40.0–47.0)

15 (19.0)

43.5 (41.0–48.6)

10 (38.5)

40.0 (34.9–44.0)

41 (33.1)

- LVDs (mm)

 - N missing

25.0 (23.0–28.0)

66 (28.8)

25.0 (23.0–30.0)

15 (19.0)

27.0 (25.0–28.0)

10 (38.5)

24.0 (22.0–28.0)

41 (33.1)

- LAD (mm)

 - N missing

34.0 (30.0–40.0)

69 (30.1)

33.5 (28.0–38.0)

15 (19.0)

36.0 (33.5–40.4)

10 (38.5)

34.0 (30.0–40.3)

44 (35.5)

- LVEF (%)

 - N missing

66.0 (61.0–70.6)

81.0 (35.4)

66.5 (61.5–70.0)

19 (24.1)

65.0 (61.0–70.1)

11 (42.3)

66.0 (60.0–72.9)

51 (41.1)

- RVDd (mm)

 - N missing

35.0 (29.0–39.0)

140 (61.1)

33.0 (25.0–37.0)

49 (62.0)

33.5 (29.0–36.0)

16 (61.5)

37.0 (31.0–41.2)

75 (60.5)

- RAD (mm)

 - N missing

40.0 (34.5–45.0)

153 (66.8)

37.0 (34.0–41.0)

48 (60.8)

37.0 (33.0–46.5)

16 (61.5)

44.0 (39.0–46.0)

89 (71.8)

- IVCd (mm)

 - N missing

13.0 (11.0–17.0)

73 (31.9)

12.0 (10.0–14.0)

18 (22.8)

14.0 (11.0–14.6)

9 (34.6)

15.0 (12.0–19.0)

46 (37.1)

- TAPSE (mm)

 - N missing

18.0 (15.0–22.0)

128 (55.9)

22.0 (19.0–25.2)

51 (64.6)

21.0 (17.8–23.5)

16 (61.5)

15.3 (13.0–19.8)

61 (49.2)

- TRV (m/sec)

 - N missing

3.4 (3.0–3.8)

196 (85.6)

2.5 (2.4–3.0)

70 (88.6)

3.3 (3.3–3.3)

25 (96.2)

3.7 (3.4–4.0)

101 (81.5)

- TRPG (mmHg)

 - N missing

43.2 (32.0–61.3)

99 (43.2)

27.7 (22.0–36.0)

37 (46.8)

38.0 (37.5–41.8)

17 (65.4)

57.0 (46.0–77.0)

45 (36.3)

Pulmonary function tests

- VC (L)

 - N missing

2.2 (1.6–2.6)

91 (39.7)

2.2 (1.9–2.8)

26 (32.9)

1.9 (1.6–2.3)

9 (34.6)

2.1 (1.4–2.5)

56 (45.2)

- VC (% predicted)

 - N missing

82.1 (59.9–97.7)

91 (39.7)

89.6 (73.0–100.7)

26 (32.9)

77.0 (60.7–84.5)

9 (34.6)

76.6 (55.8–97.1)

56 (45.2)

- FVC (L)

 - N missing

2.1 (1.6–2.6)

89 (38.9)

2.2 (1.8–2.8)

24 (30.4)

1.8 (1.6–2.2)

9 (34.6)

2.1 (1.4–2.5)

56 (45.2)

- FVC (% predicted)

 - N missing

79.6 (71.8–85.5)

90 (39.3)

80.2 (74.4–86.4)

24 (30.4)

77.4 (68.9–83.2)

10 (38.5)

78.6 (69.0–85.6)

56 (45.2)

- FEV1/FVC (%)

 - N missing

82.1 (59.9–97.7)

91 (39.7)

89.6 (73.0–100.7)

26 (32.9)

77.0 (60.7–84.5)

9 (34.6)

76.6 (55.8–97.1)

56 (45.2)

- DLCO (% predicted)

 - N missing

49.9 (36.2–65.4)

116 (50.7)

58.5 (47.6–80.3)

27 (34.2)

49.8 (41.9–58.7)

11 (42.3)

37.6 (26.8–52.5)

78 (62.9)

- DLCO/VA (% predicted)

 - N missing

64.0 (50.1–77.2)

118 (51.5)

68.2 (56.6–83.1)

29 (36.7)

69.5 (62.6–82.2)

11 (42.3)

51.5 (40.7–70.2)

78 (62.9)

Treatment before the baseline

- None

189 (82.5)

60 (75.9)

21 (80.8)

108 (87.1)

- Glucocorticoid

12 (5.2)

4 (5.1)

0 (0)

8 (6.5)

- IVCY

18 (7.9)

8 (10.1)

2 (7.7)

8 (6.5)

- Tacrolimus

9 (3.9)

3 (3.8)

4 (15.4)

2 (1.6)

- Methotrexate

7 (3.1)

6 (7.6)

0 (0)

1 (0.8)

- Azathioprine

3 (1.3)

1 (1.3)

1 (3.8)

1 (0.8)

- Cyclosporine A

3 (1.3)

1 (1.3)

0 (0)

2 (1.6)

- Tocilizumab

2 (0.9)

0 (0)

0 (0)

2 (1.6)

- Mycophenolate mofetil

1 (0.4)

1 (1.3)

0 (0)

0 (0)

- Belimumab

1 (0.4)

1 (1.3)

0 (0)

0 (0)

- Infliximab

1 (0.4)

0 (0)

0 (0)

1 (0.8)

- Rituximab

1 (0.4)

0 (0)

1 (3.8)

0 (0)

- Nintedanib

2 (0.9)

1 (1.3)

0 (0)

1 (0.8)

- Alprostadil

8 (3.5)

2 (2.5)

2 (7.7)

4 (3.2)