Background
Methods
Patients
Methods
Statistical analysis
Results
Demographic data and clinical manifestations
Patient number | |
---|---|
Gender | |
Female | 325 (79.1%) |
Male | 86 (20.9%) |
Age at onset (years) | 23.0 (18.0, 30.0)a
|
Duration of disease at first admission (months) | 21.0 (6.0, 60.0)a
|
Constitutional findings | |
Fever | 128 (31.1%) |
Malaise | 122 (29.7%) |
Weight loss | 82 (20.0%) |
Symptoms | |
Arthralgia | 37 (9.0%) |
Skin rash | 32 (7.8%) |
Vascular findings | |
Bruit of carotid arteries | 257 (62.5%) |
Hypertension | 209 (50.9%) |
Asymmetric pulsation | 158 (24.6%) |
Claudication | |
Upper limbs | 60 (14.6%) |
Lower limbs | 57 (13.9%) |
Gangrene | 2 (0.5%) |
Cardiac findings | |
Valvar lesions | 201 (48.9%) |
Left ventricle hypertrophy on ECG | 58 (14.4%) |
Heart failure | 61 (14.8%) |
Pericardial effusion | 30 (7.3%) |
Myocardial lesions | 26 (6.3%) |
Angina | 14 (3.4%) |
Myocardial infarction | 3 (0.7%) |
Renal abnormalities | |
Nephrotic-range proteinuria | 13 (3.2%) |
Renal dysfunction | 47 (11.4%) |
Neurological symptoms | |
Headache | 125 (30.4%) |
Ischemic stroke | 22 (5.4%) |
Syncope | 49 (11.9%) |
Ophthalmoscope findings | |
Hypertensive retinopathy | 31/165b (18.8%) |
TAK-related retinopathy | 23/165b (13.9%) |
Ischemic changes | 11/165b (6.7%) |
Vasculitis presentations | 11/165b (6.7%) |
Others | 3/165b (1.8%) |
Angiographic classification | |
Type I | 91 (22.1%) |
Type IIa | 16 (3.9%) |
Type IIb | 16 (3.9%) |
Type III | 12 (2.9%) |
Type IV | 26 (6.3%) |
Type V | 250 (60.8%) |
Laboratory findings | |
Level of ESR (mm/h) | 26.0 (11.0, 65.0)a
|
Level of CRP (mg/L) | 12.8 (3.4, 44.8)a
|
Level of hs-CRP (mg/L) | 8.8 (2.0, 12.5)a
|
Level of WBC (109/L) | 8.2 (6.6, 11.0)a
|
Level of Hgb (g/L) | 121 (108, 135)a
|
Features of vessel involvement and distributions
Arteries | Any arterial lesion | Stenosis | Occlusion | Dilatation | Aneurysm |
---|---|---|---|---|---|
Subclavian artery | 328 (79.8%) | 232 (56.4%) | 130 (31.6%) | 11 (2.7%) | 5 (1.2%) |
Left | 283 (68.9%) | 185 (45.0%) | 110 (26.8%) | 4 (1.0%) | 3 (0.7%) |
Right | 223 (54.2%) | 145 (35.3%) | 58 (14.1%) | 8 (1.9%) | 3 (0.7%) |
Carotid artery | 325 (79.1%) | 241 (58.6%) | 102 (24.8%) | 16 (3.9%) | 3 (0.7%) |
Left common | 291 (70.8%) | 175 (42.6%) | 82 (20.0%) | 6 (1.5%) | 0 |
Right common | 266 (64.7%) | 156 (38.0%) | 53 (12.9%) | 9 (2.2%) | 3 (0.7%) |
Left internal | 85 (20.7%) | 56 (13.6%) | 19 (4.6%) | 5 (1.2%) | 0 |
Right internal | 94 (22.9%) | 61 (14.8%) | 15 (3.6%) | 7 (1.7%) | 0 |
Left external | 59 (14.4%) | 36 (8.8%) | 14 (3.4%) | 2 (0.5%) | 0 |
Right external | 55 (13.4%) | 38 (9.2%) | 9 (2.2%) | 1 (0.2%) | 0 |
Renal artery | 201 (48.9%) | 182 (44.3%) | 43 (10.5%) | 6 (1.5%) | 2 (0.5%) |
Left | 157 (38.2%) | 143 (34.8%) | 24 (5.8%) | 5 (1.2%) | 1 (0.2%) |
Right | 149 (36.3%) | 133 (32.4%) | 24 (5.8%) | 1 (0.2%) | 1 (0.2%) |
Abdominal aorta | 158 (38.4%) | 134 (32.6%) | 12 (2.9%) | 16 (3.9%) | 10 (2.4%) |
Vertebral artery | 118 (28.7%) | 82 (20.0%) | 46 (11.2%) | 10 (2.4%) | 1 (0.2%) |
Left | 94 (22.9%) | 62 (15.1%) | 34 (8.3%) | 6 (1.5%) | 1 (0.2%) |
Right | 81 (19.7%) | 55 (13.4%) | 23 (5.6%) | 10 (2.4%) | 0 |
Mesenteric artery | 116 (29.7%) | 90 (21.9%) | 33 (8.0%) | 1 (0.2%) | 0 |
Thoracic aorta | 86 (17.5%) | 72 (17.5%) | 0 | 14 (3.4%) | 3 (0.7%) |
Innominate artery | 81 (19.7%) | 61 (14.8%) | 16 (3.9%) | 8 (1.9%) | 1 (0.2%) |
Iliacofemoral artery | 65 (15.8%) | 43 (10.5%) | 18 (4.4%) | 0 | 4 (1.0%) |
Left | 53 (12.9%) | 32 (7.8%) | 15 (3.6%) | 0 | 3 (0.7%) |
Right | 54 (13.1%) | 36 (8.8%) | 11 (2.7%) | 0 | 2 (0.5%) |
Ascending aorta | 39 (9.5%) | 4 (1.0%) | 0 | 37 (9.0%) | 3 (0.7%) |
Aortic arch | 32 (7.8%) | 25 (6.1%) | 0 | 7 (1.7%) | 1 (0.2%) |
Intracranial arterya
| 31 (7.5%) | 29 (7.1%) | 2 (0.5%) | 0 | 0 |
Pulmonary arteryb
| 31 (7.5%) | 17 (4.1%) | 14 (3.4%) | 1 (0.2%) | 0 |
Left | 22 (5.4%) | 12 (2.9%) | 6 (1.5%) | 1 (0.2%) | 0 |
Right | 27 (6.6%) | 12(2.9%) | 11 (2.7%) | 1 (0.2%) | 0 |
Coronary arteryc
| 15 (3.6%) | 14 (3.4%) | 4 (1.0%) | 0 | 0 |
Left | 13 (3.2%) | 10 (2.4%) | 3 (0.7%) | 0 | 0 |
Right | 7 (1.7%) | 7 (1.7%) | 1 (0.2%) | 0 | 0 |
Arteries | This study (n = 411) | Mwipatayi et al. [12] (n = 272) | Lee et al. [22] (n = 204) | Schmidt et al. [21] (n = 126) | Vanoli et al. [24] (n = 104) | Arnaud et al. [23] (n = 82) | Freitas et al. [20] (n = 52) | |||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
Left | Right | Left | Right | Left | Right | Left | Right | Left | Right | |||
Subclavian artery | 328 (79.8%) | ND | 67.1% | 55.2% | 66.3% | 41.0% | 65.6% | 52.5% | 68.3% | 50.0% | 60.6% | 40.4% |
Stenosis | 232 (56.4%) | ND | 26.0% | 24.6% | 43.3% | 36.2% | 42.6% | 29.5% | ND | 25.0% | 26.9% | |
Occlusion | 130 (31.6%) | ND | 34.8% | 14.3% | 29.8% | 4.8% | 23.0% | 23.0% | ND | 25.0% | 5.8% | |
Dilatation | 11 (2.7%) | ND | 0.5% | 4.4% | ND | ND | 0 | 0 | ND | 0 | 5.8% | |
Aneurysm | 5 (1.2%) | ND | 0 | 1.0% | 1.6% | 0 | ND | 1.9% | 1.9% | |||
Carotid artery | 325 (79.1%) | 83 (30.5%) | 72.1%f
| 63.7%f
| 50.9% | 41.7% | 44.3% | 36.1% | 59.8%f
| 52.4%f
| 57.3% | 40.4% |
Stenosis/occlusion | 260 (63.3%) | 59 (21.7%) | ND | ND | ND | ND | ND | ND | ND | ND | ND | |
Stenosis | 241 (58.6%) | ND | 33.3%f
| 32.3%f
| 41.7% | 37.0% | 37.7% | 23.0% | ND | 30.8% | 23.1% | |
Occlusion | 102 (24.8%) | ND | 21.1%f
| 9.8%f
| 10.2% | 5.6% | 4.9% | 6.6% | ND | 9.6% | 9.6% | |
Dilatation | 16 (3.9%) | ND | 1.0%f
| 1.5%f
| ND | ND | 0 | 3.3% | ND | 1.9% | 0 | |
Aneurysm | 2 (0.5%) | 17 (6.3%) | 0.9% | 0.9% | 1.6% | 3.3% | ND | 5.8% | 1.9% | |||
Aneurysm and stenosis/occlusion | 1 (0.2%) | 7 (2.6%) | ND | ND | ND | ND | ND | ND | ND | ND | ND | |
Renal artery | 201 (48.9%) | ND | 32.2% | 31.7% | 18.7% | 20.9% | 34.4% | 29.5% | 14.6% | 15.9% | 34.6% | 36.5% |
Stenosis | 182 (44.3%) | ND | 25.2% | 25.2% | 16.5% | 19.8% | 31.2% | 23.0% | ND | 15.4% | 25.0% | |
Occlusion | 43 (10.5%) | ND | 6.0% | 4.5% | 4.4% | 2.2% | 3.3% | 6.6% | ND | 9.6% | 3.8% | |
Dilatation | 6 (1.5%) | ND | 0 | 0 | ND | ND | 0 | 0 | ND | 0 | 0 | |
Aneurysm | 2 (0.5%) | ND | 0 | 0 | 0 | 0 | 0 | 0 | ND | 0 | 0 | |
Abdominal aorta | 158 (38.4%) | 186 (68.4%) | 63.2% | 23.7%c
| 27.4%d
| 39.3% | 43.9% | 63.5% | ||||
Stenosis/occlusion | 140 (34.1%) | 115 (42.3%) | ND | ND | ND | ND | ND | ND | ||||
Stenosis | 134 (32.6%) | ND | 38.3% | 20.4%c
| 25.3%d
| 29.5% | ND | 26.9% | ||||
Occlusion | 12 (2.9%) | ND | 4.0% | 1.1%c
| 2.1%d
| 6.6% | ND | 1.9% | ||||
Aneurysm | 6 (1.4%) | 41 (15.1%) | 6.0% | 2.2%c
| 1.1%d
| 3.3% | ND | 7.7% | ||||
Dilatation | 0 | ND | ND | ND | ND | ND | 11.5% | |||||
Aneurysm and stenosis/occlusion | 4 (1.0%) | 30 (11.0%) | ND | ND | ND | ND | ND | ND | ||||
Vertebral artery | 118 (28.7%) | ND | ND | 18.5% | 13.0% | 13.3% | 11.7% | 28.0% | 15.9% | 25.0% | 11.5% | |
Stenosis | 82 (20.0%) | ND | ND | 15.7% | 9.3% | 8.3% | 8.3% | ND | 13.5% | 5.8% | ||
Occlusion | 46 (11.2%) | ND | ND | 2.8% | 3.7% | 5.0% | 1.7% | ND | 3.8% | 5.8% | ||
Dilatation | 10 (2.4%) | ND | ND | ND | ND | 0 | 1.7% | ND | 1.9% | 0 | ||
Aneurysm | 1 (0.2%) | ND | ND | 0 | 0 | 0 | 0 | ND | 0 | 0 | ||
Mesenteric artery | 116 (29.7%) | 101 (37.1%) | 22.8%a
| 3.5%b
| 24.7%a
| 6.9%b
| 31.6%a
| 9.4%b
| ND | 28.8%a
| 3.8%b
| |
Stenosis/occlusion | 116 (29.7%) | 92 (33.8%) | ND | ND | ND | ND | ND | ND | ND | ND | ND | |
Stenosis | 90 (21.9%) | ND | 14.4%a
| 0.5%b
| 18.0%a
| 2.3%b
| 24.6%a
| 5.7%b
| ND | 15.4%a
| 3.8%b
| |
Occlusion | 33 (8.0%) | ND | 5.9%a
| 2.0%b
| 6.7%a
| 4.6%b
| 7.0%a
| 1.9%b
| ND | 11.5%a
| 0b
| |
Dilatation | 1 (0.2%) | ND | 1.0%a
| 1.0%b
| ND | ND | 0a
| 1.9%b
| ND | 0a
| 0b
| |
Aneurysm | 0 | 9 (3.3%) | 0a
| 0b
| 0a
| 0b
| ND | 0a
| 0b
| |||
Thoracic aorta | 86 (17.5%) | 158 (58.1%) | 57.2% | 19.1% | 11.1% | 40.2% | 34.6% | |||||
Stenosis/occlusion | 72 (17.5%) | 98 (36.0%) | ND | 18.2% | ND | ND | ND | |||||
Stenosis | 72 (17.5%) | ND | 22.9% | 18.2% | 7.4% | ND | 9.6% | |||||
Occlusion | 0 | ND | 0 | 0 | 0 | ND | 0 | |||||
Dilatation | 14 (3.4%) | ND | 3.0% | ND | 1.9% | ND | 9.6% | |||||
Aneurysm | 2 (0.5%) | 38 (14.0%) | 0.9% | 1.9% | ND | 3.8% | ||||||
Aneurysm and stenosis/occlusion | 1 (0.2%) | 22 (8.1%) | ND | ND | ND | ND | ND | |||||
Innominate artery | 81 (19.7%) | 43 (10.5%) | 46.8% | 25.5% | 8.8% | 28.0% | 25.0% | |||||
Stenosis/occlusion | 74 (18.0%) | 22 (8.1%) | ND | ND | ND | ND | ND | |||||
Stenosis | 61 (14.8%) | ND | 16.3% | 18.9% | 8.8% | ND | 11.5% | |||||
Occlusion | 16 (3.9%) | ND | 3.0% | 6.6% | 0 | ND | 1.9% | |||||
Dilatation | 8 (1.9%) | ND | 4.0% | ND | 1.8% | ND | 7.7% | |||||
Aneurysm | 1 (0.2%) | 16 (5.9%) | 0.9% | 0 | ND | 1.9% | ||||||
Aneurysm and stenosis/occlusion | 0 | 5 (1.8%) | ND | ND | ND | ND | ND | |||||
Iliacofemoral artery | 65 (15.8%) | 74 (27.2%) | 13.3%g
| 14.8%g
| 13.5%g
| 13.5%g
| 19.7%g
| 18.9%g
| 12.2%g
| 18.3%g
| 23.1%g
| 23.1%g
|
Stenosis | 43 (10.5%) | ND | 6.9%g
| 8.9%g
| 10.1%g
| 10.1%g
| 9.8%g
| 6.6%g
| ND | 11.5%g
| 9.6%g
| |
Occlusion | 18 (4.4%) | ND | 4.9%g
| 3.4%g
| 4.5%g
| 2.2%g
| 8.2%g
| 9.8%g
| ND | 5.8%g
| 5.8%g
| |
Dilatation | 0 | ND | 0.5%g
| 0g
| ND | ND | 1.6%g
| 1.6%g
| ND | 0g
| 0g
| |
Aneurysm | 4 (1.0%) | ND | 0g
| 2.2%g
| 0g
| 0g
| ND | 0g
| 0g
| |||
Ascending aorta | 39 (9.5%) | 65 (23.9%) | 47.8% | 9.1% | ND | ND | 30.8% | |||||
Stenosis | 3 (0.7%) | 15 (5.5%) | 0 | 2.7% | ND | ND | 1.9% | |||||
Dilatation | 37 (9.0%) | ND | 25.4% | ND | ND | ND | 19.2% | |||||
Aneurysm | 2 (0.5%) | 43 (15.8%) | 6.4% | ND | ND | 3.8% | ||||||
Aneurysm and stenosis | 1 (0.2%) | 7 (2.6%) | ND | ND | ND | ND | ND | |||||
Aortic arch | 32 (7.8%) | 90 (33.1%) | 37.9% | 4.5% | 10.3% | 37.8% | 19.2% | |||||
Stenosis | 25 (6.1%) | 45 (16.5%) | 0.5% | 2.7% | 5.2% | ND | 1.9% | |||||
Dilatation | 7 (1.7%) | ND | 3.4% | ND | 5.2% | ND | 9.6% | |||||
Aneurysm | 1 (0.2%) | 36 (13.2%) | 1.8% | 0 | ND | 1.9% | ||||||
Aneurysm and stenosis | 0 | 9 (3.3%) | ND | ND | ND | ND | ND | |||||
Pulmonary artery | 31/45e (68.9%) | 24 (8.8%) | 13.4% | 6/18e (33.3%) | ND | ND | ND | |||||
Stenosis/occlusion | 26/45e (57.8%) | 13 (4.8%) | ND | ND | ND | ND | ND | |||||
Stenosis | 17/45e (37.8%) | ND | 4.0% | 6/18e (33.3%) | ND | ND | ND | |||||
Occlusion | 14/45e (31.1%) | ND | 2.0% | 2/18e (11.1%) | ND | ND | ND | |||||
Dilatation | 1/45e (2.2%) | ND | 2.0% | ND | ND | ND | ND | |||||
Aneurysm | 0 | 6 (2.2%) | 0 | ND | ND | ND | ||||||
Aneurysm and stenosis/occlusion | 0 | 5 (1.8%) | ND | ND | ND | ND | ND | |||||
Coronary artery | 15/42e (35.7%) | ND | 31/49e (63.3%) | 4/18e (22.2%) | ND | ND | ND | |||||
Stenosis | 14/42e (33.3%) | ND | 26/49e (53.1%) | 4/18e (22.2%) | ND | ND | ND | |||||
Occlusion | 4/42e (9.5%) | ND | 2/49e (4.1%) | 1/18e (5.6%) | ND | ND | ND | |||||
Dilatation | 0 | ND | 3/49e (6.1%) | ND | ND | ND | ND | |||||
Aneurysm | 0 | ND | 0 | ND | ND | ND |
China/this study | Turkey [16] | Korea [22] | US [21] | China [15] | Mexico [34] | Korea [25] | India [26] | Japan [26] | Thailand [13] | |
---|---|---|---|---|---|---|---|---|---|---|
n
| 411 | 248 | 204d
| 126e
| 125 | 110 | 108 | 102 | 79 | 63 |
Disease durations (months) | 21.0 (6.0, 60.0)a
| 34.2 (0, 240)b
| ND | 13.3 (6.1, 35.5)a
| 19 (0.5, 160)b
| ND | 13.9 ± 10.1 (1, 60)b
| ND | 24.3 ± 11.5c
| ND |
Type I (%) | 22.1 | 32 | 11.1 | 20 | 40 | 19 | 36.1 | 6.9 | 24.1 | 0 |
Type IIa (%) | 3.9 | 6.9 | 8.6 | 6 | 4.8 | 3 | 2.8 | 1 | 11.4 | 0 |
Type IIb (%) | 3.9 | 3.2 | 14.1 | 7 | 1.6 | 4 | 4.6 | 5.9 | 10.1 | 11.1 |
Type III (%) | 2.9 | 3.2 | 4.0 | 5 | 2.4 | 4 | 7.4 | 2.9 | 0 | 3.2 |
Type IV (%) | 6.3 | 3.7 | 7.6 | 5 | 20.8 | 2 | 15.7 | 28.4 | 1.3 | 19 |
Type V (%) | 60.8 | 51 | 54.5 | 57 | 30.4 | 69 | 33.3 | 54.9 | 53.2 | 66.7 |
TAK-related surgical procedures and interventions
Category of operations/interventions | Patient numbers | % |
---|---|---|
Operations on cervical vessels | 53 | 12.9 |
Bypass between aorta and carotid artery | 21 | 5.1 |
Bypass between carotid and subclavian arteries | 11 | 2.7 |
Bypass between aorta and subclavian arteries | 6 | 1.5 |
Angioplasty and/or stenting in carotid arteries | 6 | 1.5 |
Angioplasty and/or stenting in subclavian arteries | 4 | 1.0 |
Angioplasty and/or stenting in innominate arteries | 3 | 0.7 |
Bypass between innominate and carotid arteries | 1 | 0.2 |
Bypass between innominate and subclavian arteries | 1 | 0.2 |
Operations to reduce renal hypertension | 33 | 8.0 |
Angioplasty and/or stenting in renal arteries | 25 | 6.1 |
Bypass between aorta and renal arteries | 4 | 1.0 |
Nephrectomy | 4 | 1.0 |
Operations on aortic and iliacofemoral stenosis or occlusion | 20 | 4.9 |
Angioplasty and/or stenting in aorta | 12 | 2.9 |
Aorta bypass grafting | 5 | 1.2 |
Bypass between aorta and bilateral iliac arteries | 1 | 0.2 |
Bypass between aorta and bilateral femoral arteries | 1 | 0.2 |
Angioplasty and/or stenting in common iliac arteries | 1 | 0.2 |
Operations on valvar lesions and coronary artery | 9 | 2.2 |
Cardiac valve replacement | 5 | 1.2 |
Angioplasty and/or stenting in coronary arteries | 3 | 0.7 |
Coronary artery bypass grafting | 1 | 0.2 |
Repair on aortic aneurysm | 2 | 0.5 |
Ascending aorta and aortic arch | 1 | 0.2 |
Thoracic and abdominal aorta | 1 | 0.2 |
Rebuilding circulation in upper limbs | 1 | 0.2 |
Bypass between axillary arteries | 1 | 0.2 |
Rebuilding circulation in mesenteric arteries | 1 | 0.2 |
Mesenteric artery bypass | 1 | 0.2 |
Summary | ||
Bypass operations | 54 | 13.1 |
Percutaneous transluminal angiography (PTA) | 47 | 11.4 |
PTA in renal arteries | 25 | 6.1 |
Stenting | 35 | 8.5 |
Other operations | 11 | 2.7 |