Ross
27 KiB
27 KiB
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| Takayasu Arteritis | d35eb6f3-bfd3-4121-8781-325a93ccc197 |
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Cardiac | 1f530233-4bd8-423a-9dcc-253d9f03082a | 12 | 12/19/24 | Takayasu Arteritis | Cardiac, Diagnosis, Aorta, Takayasu Arteritis | Takayasu Arteritis | STATdx | Takayasu Arteritis | DX | true | 1 |
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- "Cardiac"
- "Diagnosis"
- "Aorta"
- "Takayasu Arteritis"
KEY FACTS
-
Terminology
- Takayasu arteritis (TA)
- Pulseless disease
- Chronic granulomatous vasculitis of large vessels
-
Imaging
- Best diagnostic clue: Wall thickening of large vessels - Thoracic aorta and branches - Pulmonary artery involvement is less common
- NECT: Aortic wall thickening
- CECT: Aortic wall thickening and enhancement - Stenosis, occlusion, aneurysm
- MRA: Aortic narrowing, dilatation
- Angiography: 4 types classified by location
- PET/CT is used for treatment monitoring
- Complications - Stenosis > occlusion - Aneurysm - Dissection
-
Top Differential Diagnoses
- Giant cell arteritis
- Aortic coarctation
-
Pathology
- Autoimmune etiology is suspected
- Specific types of human leukocyte antigen are common among patients
-
Clinical Issues
- Disease stages - Early or prepulseless phase - Vascular inflammatory phase - Late quiescent occlusive or pulseless phase - Triphasic disease in minority of patients
- F:M = 8:1
- Heart failure is most common cause of death
- Treatment - Corticosteroids, angioplasty, surgical bypass
TERMINOLOGY
-
Abbreviations
- Takayasu ar****teritis (TA)
-
Synonyms
- Pulseless disease
-
Definitions
- Chronic granulomatous vasculitis of large vessels
IMAGING
-
General Features
-
Best diagnostic clue
- Wall thickening of large vessels -
Location
- **Thoracic aorta****and****branches** - **Left subclavian artery** is most commonly affected - Ostial stenoses or occlusion of arch vessels - Pulmonary artery (PA) involvement is less common -
Evidence of medium or large vessel involvement on imaging is absolute requirement for diagnosis of TA
-
-
Radiographic Findings
-
Radiography
- Irregular or dilated descending thoracic aorta - Diminished pulmonary vessels and rib notching
-
-
CT Findings
-
NECT
- Vessel wall thickening, iso-/hyperdense to muscle -
CECT
- **Vessel wall thickening****and****enhancement** - **Stenosis, occlusion, aneurysm** - 95% sensitivity and 100% specificity for diagnosis of TA - Delayed/venous phase: Double ring enhancement pattern - Hyperenhanced outside ring: Active inflammation of media and adventitia - Poor enhancing inside ring: Swelling of intima - Can be used to assess treatment response
-
-
MR Findings
-
T1WI
- **Wall thickening: Aorta****and****branches** - Wall edema can be seen in STIR images -
T1WI C+
- **Enhancement of thickened vessel wall** -
MRA
- Focal/diffuse narrowing of aorta and branches - Aortic dilatation (ascending > descending) - Stenosis > occlusion - Aortic regurgitation, dissection, aneurysm -
Disease activity - Active disease: Wall edema and delayed enhancement can be seen - Poor and inconsistent correlation with disease activity - Development of new lesion in previously unaffected vascular territory is evidence of active disease/progression - Evaluate for new lesions in other territories
-
-
Ultrasonographic Findings
-
Grayscale ultrasound
- Vascular wall thickening, dilation, stenosis - Diffuse arterial wall thickening: Macaroni sign - Carotid intima-media thickness > 1 mm is considered to be inflammatory vascular disease -
Color Doppler
- Luminal stenosis, change in blood flow patterns - 81% sensitivity and 100% specificity for diagnosing TA
-
-
Angiographic Findings
- Early: Aortic wall thickening, rarely, stenosis
- Late: Stenosis, occlusion, aneurysm; 4 types - I: Aortic arch branches - II: Thoracic aorta (a: Ascending, B: Descending) and branch vessels - III: Descending thoracic and abdominal aorta ± renal arteries; may have atypical coarctation - IV: Abdominal aortic ± renal arteries - V: Entire aorta and its branches
-
Nuclear Medicine Findings
-
PET
- FDG uptake; ranges from low grade to intense - Treatment monitoring: Multiple qualitative and quantitative methods
-
-
Imaging Recommendations
-
Protocol advice
- Multiplanar reconstructions for stenosis
-
DIFFERENTIAL DIAGNOSIS
-
- Affects large vessels in older patients (> 50 years)
-
- Infections (tuberculosis, syphilis, HIV, bacterial)
- Atherosclerosis; thromboembolism
- Genetic disorders: Marfan, Ehlers-Danlos IV, Loeys-Dietz, Grange
- Congenital: Coarctation, Turner syndrome, Williams syndrome
- Unknown etiology: Fibromuscular dysplasia, segmental arterial mediolysis
-
- Large vessel vasculitis seen in 30% of these patients
- Proximal PA aneurysms are common
-
Other Causes of Aortitis
- Ankylosing spondylitis, rheumatoid arthritis, Cogan syndrome, relapsing polychondritis, IgG4-related disease
PATHOLOGY
-
General Features
-
Etiology
- Autoimmune etiology is suspected - Infectious triggers suspected: Mycobacterium tuberculosis - Suspected association with active or latent TB -
Genetics
- Specific types of human leukocyte antigen are common - Strong association with HLA-B52
-
-
Gross Pathologic & Surgical Features
- Wall thickening of large vessels
-
Microscopic Features
- Granulomatous inflammation of arterial wall - Intimal proliferation; fibrosis of media and adventitia
CLINICAL ISSUES
-
Presentation
-
Most common signs/symptoms
- **Early or prepulseless phase** - Low-grade fever, malaise, weight loss, fatigue - **Vascular inflammatory phase** - Vascular insufficiency - Symptoms are minimized by collateral formation - **Late quiescent occlusive or pulseless phase** - Diminished/absent pulses, vascular bruits, claudication - Blood pressure discrepancies in upper extremity - Subclavian steal syndrome - Hypertension, aortic regurgitation - Neurologic symptoms: Headache, dizziness, seizures, stroke - Ocular symptoms (ranging 8-68%): Hypertensive retinopathy or Takayasu retinopathy (due to hypoperfusion) - Triphasic pattern is seen in minority of patients - Disease is usually recurrent; phases may coexist - Interval between early and late phases is variable - **Cardiac involvement** - Myocardial ischemia: 84% of asymptomatic patients - Can cause angina, myocardial infraction, or sudden cardiac death - Coronary involvement is independent predictor of poor long-term outcomes - Type 1 (most common): Stenosis or occlusion of coronary ostia and proximal coronary artery - Type 2: Diffuse or focal coronary arteritis (skip lesions) - Type 3: Coronary aneurysms -
Other signs/symptoms
- Pulmonary hypertension when PA is involved - Increases risk of early mortality
-
-
Demographics
-
Age
- Most common in 2nd and 3rd decades of life -
Sex
- F:M = 8:1 -
Epidemiology
- Most common in Asia - Affects 6 out of 1,000 persons worldwide - Annual incidence in USA: 2-3
-
-
Natural History & Prognosis
- Congestive heart failure is most common cause of death
- Hypertension is poor prognostic factor
-
Treatment
- Corticosteroids are 1st-line treatment; cyclophosphamide and methotrexate are 2nd line
- Angioplasty, surgical bypass, or stent placement for stenosis and occlusion
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References
Selected References
- Somashekar A et al: Updates in the diagnosis and management of Takayasu's arteritis. Postgrad Med. 135(sup1):14-21, 2023
- Grayson PC et al: 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis. Arthritis Rheumatol. 74(12):1872-80, 2022
- Jia S et al: Application progress of multiple imaging modalities in Takayasu arteritis. Int J Cardiovasc Imaging. 37(12):3591-601, 2021
- Chatterjee S et al: Clinical diagnosis and management of large vessel vasculitis: Takayasu arteritis. Curr Cardiol Rep. 16(7):499, 2014
- Khandelwal N et al: Multidetector CT angiography in Takayasu arteritis. Eur J Radiol. 77(2):369-74, 2011
- Restrepo CS et al: Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta. Radiographics. 31(2):435-51, 2011
- Pipitone N et al: Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford). 47(4):403-8, 2008
- Desai MY et al: Delayed contrast-enhanced MRI of the aortic wall in Takayasu's arteritis: initial experience. AJR Am J Roentgenol. 184(5):1427-31, 2005
- Tso E et al: Takayasu arteritis: utility and limitations of magnetic resonance imaging in diagnosis and treatment. Arthritis Rheum. 46(6):1634-42, 2002
Tables
Classification Criteria for Takayasu Arteritis: 2022 ACR/EULAR
| Absolute Requirements | |
| Age ≤ 60 | |
| Evidence of vasculitis on imaging | |
| Additional Clinical Criteria | |
| Female sex | +1 |
| Angina or ischemic cardiac pain | +2 |
| Claudication | +2 |
| Vascular bruit | +2 |
| Diminished pulse in upper extremity | +2 |
| Carotid artery abnormality | +2 |
| Systolic BP difference in arms ≥ 20 mmHg | +1 |
| Additional Imaging Criteria | |
| Number of affected artery territory | |
| 1 arterial territory | +1 |
| 2 arterial territories | +2 |
| 3 or more arterial territories | +3 |
| Symmetric involvement of paired arteries | +1 |
| Abdominal aorta involvement with renal or mesenteric involvement | +3 |
| Score of ≥ 5 points = Takayasu arteritis |
Anatomy
Cervical Carotid Arteries
Brain/ANATOMY:6eafa14e-2538-41b5-850c-29c41f38b970
Aortic Arch and Great Vessels
Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
Pulmonary Vessels
Chest/ANATOMY:551f4b47-fac1-44f9-b800-09ce766fedd9
Vessels, Lymphatic System, and Nerves, Abdominal
Gastrointestinal/ANATOMY:0c38fd49-88e7-4272-960f-b17a194ce0cc
Carotid Space
Head and Neck/ANATOMY:627bdee1-4bde-46f2-b93d-958882586337
Aorta and Inferior Vena Cava
Ultrasound/ANATOMY:eed39e4d-478b-45d3-9406-1eace6e9eef1
Abdominal Aorta and Visceral Vasculature Anatomy
Vasculature/ANATOMY:341c0af5-bfe8-4755-a401-39b7b9f2d9b9
Cases
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Images
Selected Images
Axial CECT in a patient with Takayasu arteritis (TA) shows marked thickening of the wall of the ascending thoracic aorta 
. The thoracic aorta and its branches, particularly the left subclavian artery, are the most commonly affected vessels in TA.
Axial CECT in a patient with Takayasu arteritis (TA) shows marked thickening of the wall of the ascending thoracic aorta . The thoracic aorta and its branches, particularly the left subclavian artery, are the most commonly affected vessels in TA.
Axial T1 MR in a patient with TA demonstrates thickening of the wall of the ascending thoracic aorta 
and the pulmonary trunk . Vessel stenosis, occlusion, and aneurysm formation may complicate cases of TA.
Axial fused PET/CT in a patient with active TA demonstrates intense FDG uptake within the mediastinum adjacent to the aortic arch 
in a region of soft tissue attenuation that was present on the localization CT.
Axial fused PET/CT in the same patient shows intense FDG uptake within the mediastinum adjacent to the ascending aorta and pulmonary arteries . FDG uptake may be low grade to intense in TA, and PET/CT is an effective way of monitoring treatment response.
Composite image with axial CECT shows wall thickening 
of the proximal right brachiocephalic, left common carotid, and left subclavian arteries. There is more distal occlusion of the left common carotid and left subclavian arteries. The left subclavian artery is the most common branch vessel affected in patients with TA.
Sagittal oblique DSA in another patient with TA shows patency of the left common carotid artery with occlusion of the right brachiocephalic 
and left subclavian arteries .
Axial CECT in a patient with TA shows soft tissue attenuation and no contrast opacification within the left subclavian artery , consistent with occlusion.
Sagittal oblique DSA in the same patient with TA shows occlusion of the left subclavian artery . Note the common origin of the right brachiocephalic and left common carotid arteries.
Composite image with axial T1 MR (left) and DSA (right) shows aortic wall thickening and a focal aneurysm confirmed on subtraction aortic DSA .
. TA was confirmed on pathologic examination of the resected specimen.
Additional Images
, consistent with occlusion.
, consistent with luminal occlusion.