Table 1.
Demographic and clinical features
| Parameter | Total (n = 216) | Patients without PAI (n = 160) | Patients with PAI (n = 56) | P* |
|---|---|---|---|---|
| General information | ||||
| Female:male | 178:37 | 130:30 | 48:7 | 0.41 |
| Age at diagnosis (years, mean ± SD) | 35.19 ± 14.75 | 34.23 ± 15.05 | 37.96 ± 13.57 | 0.11 |
| Active status (n (%)) | 118 (54.63) | 91 (56.88) | 27 (48.21) | 0.63 |
| Observational period (month, quartile) | 12 (7, 25) | 12 (4.5, 24) | 11 (6.7, 27) | 0.43 |
| Clinical symptoms | ||||
| Headache/dizziness (n (%)) | 87 (40.28) | 73 (45.63) | 14 (25.00) | 0.023 |
| Weakness (n (%)) | 64 (29.63) | 53 (33.13) | 11 (19.64) | 0.16 |
| Chest pain/distress | 51 (23.61) | 27 (16.88) | 24 (42.86) | < 0.001 |
| Fever (n (%)) | 26 (12.04) | 24 (15.00) | 2 (3.57) | 0.048 |
| Amaurosis (n (%)) | 25 (11.57) | 21 (13.13) | 4 (7.14) | 0.46 |
| Weight loss (n (%)) | 20 (9.26) | 17 (10.63) | 3 (5.36) | 0.42 |
| Oral ulcer (n (%)) | 12 (5.56) | 6 (3.75) | 6 (10.71) | 0.04 |
| Cough/sputum (n (%)) | 9 (4.17) | 3 (1.88) | 6 (10.71) | 0.008 |
| Haemoptysis (n (%)) | 5 (2.31) | 2 (1.25) | 3 (5.36) | 0.092 |
| Clinical signs | ||||
| Vascular murmur (n (%)) | 68 (31.48) | 46 (28.75) | 22 (39.29) | 0.087 |
| Hypertension (n (%)) | 51 (23.61) | 35 (21.88) | 16 (28.57) | 0.19 |
| Pulselessness/weak pulse (n (%)) | 46 (21.30) | 32 (20) | 14 (25.00) | 0.34 |
| Neck pain (n (%)) | 14 (6.48) | 11 (6.87) | 3 (5.36) | 0.21 |
| Claudication (n (%)) | 13 (6.02) | 7 (4.38) | 6 (10.71) | 0.09 |
| Imaging features | ||||
| Aortic regurgitation (n (%)) | 50 (23.15) | 34 (21.25) | 16 (28.57) | 0.27 |
| Pulmonary hypertension (n (%)) | 34 (15.74) | 9 (5.63) | 28 (50.00) | < 0.001 |
| Type I | 61 (28.24) | 52 (32.5) | 9 (16.07) | 0.001 |
| 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) | |
| Laboratory results | ||||
| Haemoglobin (mean ± SD, g/L) | 117.78 ± 20.18 | 117.70 ± 20.22 | 118.04 ± 20.24 | 0.92 |
| White blood cells (mean ± SD, 109/L) | 9.29 ± 10.27 | 9.30 ± 11.05 | 9.24 ± 7.52 | 0.97 |
| Platelets (mean ± SD, 109/L) | 287.33 ± 110.05 | 297.33 ± 111.67 | 256.94 ± 99.98 | 0.02 |
| Erythrocyte sedimentation rate (mmHg) | 37.25 ± 33.84 | 38.66 ± 33.66 | 33.11 ± 34.33 | 0.31 |
| C-reactive protein (mg/L) | 20.16 ± 31.31 | 21.40 ± 31.97 | 18.30 ± 29.41 | 0.54 |
| Interleukin-6 (pg/L) | 10.16 ± 14.19 | 10.17 ± 13.95 | 10.11 ± 15.06 | 0.98 |
*P value: comparison between patients with and without pulmonary artery involvement (PAI)