Table 1 Demographic and clinical features

 Female:male

178:37

130:30

48:7

 Age at diagnosis (years, mean ± SD)

35.19 ± 14.75

34.23 ± 15.05

37.96 ± 13.57

 Active status (n (%))

118 (54.63)

91 (56.88)

27 (48.21)

 Observational period (month, quartile)

12 (7, 25)

12 (4.5, 24)

11 (6.7, 27)

 Headache/dizziness (n (%))

87 (40.28)

73 (45.63)

14 (25.00)

 Weakness (n (%))

64 (29.63)

53 (33.13)

11 (19.64)

 Chest pain/distress

51 (23.61)

27 (16.88)

24 (42.86)

 Fever (n (%))

26 (12.04)

24 (15.00)

2 (3.57)

 Amaurosis (n (%))

25 (11.57)

21 (13.13)

4 (7.14)

 Weight loss (n (%))

20 (9.26)

17 (10.63)

3 (5.36)

 Oral ulcer (n (%))

12 (5.56)

6 (3.75)

6 (10.71)

 Cough/sputum (n (%))

9 (4.17)

3 (1.88)

6 (10.71)

 Haemoptysis (n (%))

5 (2.31)

2 (1.25)

3 (5.36)

 Vascular murmur (n (%))

68 (31.48)

46 (28.75)

22 (39.29)

 Hypertension (n (%))

51 (23.61)

35 (21.88)

16 (28.57)

 Pulselessness/weak pulse (n (%))

46 (21.30)

32 (20)

14 (25.00)

 Neck pain (n (%))

14 (6.48)

11 (6.87)

3 (5.36)

 Claudication (n (%))

13 (6.02)

7 (4.38)

6 (10.71)

 Aortic regurgitation (n (%))

50 (23.15)

34 (21.25)

16 (28.57)

 Pulmonary hypertension (n (%))

34 (15.74)

9 (5.63)

28 (50.00)

 Type I

61 (28.24)

52 (32.5)

9 (16.07)

 Type IIa

12 (5.56)

9 (5.63)

3 (5.36)

 Type IIb

25 (11.57)

11 (6.88)

14 (25.00)

 Type III

12 (5.56)

12 (7.50)

0 (0)

 Type VI

12 (5.56)

10 (6.25)

2 (3.57)

 Type V

94 (43.52)

66 (41.25)

28 (50)

 Haemoglobin (mean ± SD, g/L)

117.78 ± 20.18

117.70 ± 20.22

118.04 ± 20.24

 White blood cells (mean ± SD, 10⁹/L)

9.29 ± 10.27

9.30 ± 11.05

9.24 ± 7.52

 Platelets (mean ± SD, 10⁹/L)

287.33 ± 110.05

297.33 ± 111.67

256.94 ± 99.98

 Erythrocyte sedimentation rate (mmHg)

37.25 ± 33.84

38.66 ± 33.66

33.11 ± 34.33

 C-reactive protein (mg/L)

20.16 ± 31.31

21.40 ± 31.97

18.30 ± 29.41

 Interleukin-6 (pg/L)

10.16 ± 14.19

10.17 ± 13.95

10.11 ± 15.06