444 lines
27 KiB
Markdown
444 lines
27 KiB
Markdown
---
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title: "Takayasu Arteritis"
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docid: "d35eb6f3-bfd3-4121-8781-325a93ccc197"
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authors:
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value: "Arzu Canan, MD"
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value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
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name: "Cardiac"
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name: "Aorta"
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treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
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name: "Takayasu Arteritis"
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slug: "takayasu-arteritis"
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category: "Cardiac"
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imageCount: 12
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lastUpdated: "12/19/24"
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pageDescription: "Takayasu Arteritis"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Takayasu Arteritis"
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pageTitle: "Takayasu Arteritis | STATdx"
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type: "DX"
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cases: 1
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breadcrumbs:
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- "Cardiac"
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- "Diagnosis"
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- "Aorta"
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- "Takayasu Arteritis"
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---
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# KEY FACTS
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- ## Terminology
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- Takayasu arteritis (TA)
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- Pulseless disease
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- Chronic granulomatous vasculitis of large vessels
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- ## Imaging
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- Best diagnostic clue: Wall thickening of large vessels
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- Thoracic aorta and branches
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- Pulmonary artery involvement is less common
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- NECT: Aortic wall thickening
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- CECT: Aortic wall thickening and enhancement
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- Stenosis, occlusion, aneurysm
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- MRA: Aortic narrowing, dilatation
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- Angiography: 4 types classified by location
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- PET/CT is used for treatment monitoring
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- Complications
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- Stenosis > occlusion
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- Aneurysm
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- Dissection
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- ## Top Differential Diagnoses
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- Giant cell arteritis
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- Aortic coarctation
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- ## Pathology
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- Autoimmune etiology is suspected
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- Specific types of human leukocyte antigen are common among patients
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- ## Clinical Issues
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- Disease stages
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- Early or prepulseless phase
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- Vascular inflammatory phase
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- Late quiescent occlusive or pulseless phase
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- Triphasic disease in minority of patients
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- F:M = 8:1
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- Heart failure is most common cause of death
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- Treatment
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- Corticosteroids, angioplasty, surgical bypass
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# TERMINOLOGY
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- ## Abbreviations
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- **Takayasu ar****teritis (TA)**
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- ## Synonyms
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- **Pulseless disease**
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- ## Definitions
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- Chronic granulomatous vasculitis of large vessels
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Wall thickening of large vessels
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- ### Location
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- **Thoracic aorta****and****branches**
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- **Left subclavian artery** is most commonly affected
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- Ostial stenoses or occlusion of arch vessels
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- Pulmonary artery (PA) involvement is less common
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- Evidence of medium or large vessel involvement on imaging is absolute requirement for diagnosis of TA
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- ## Radiographic Findings
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- ### Radiography
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- Irregular or dilated descending thoracic aorta
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- Diminished pulmonary vessels and rib notching
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- ## CT Findings
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- ### NECT
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- Vessel wall thickening, iso-/hyperdense to muscle
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- ### CECT
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- **Vessel wall thickening****and****enhancement**
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- **Stenosis, occlusion, aneurysm**
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- 95% sensitivity and 100% specificity for diagnosis of TA
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- Delayed/venous phase: Double ring enhancement pattern
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- Hyperenhanced outside ring: Active inflammation of media and adventitia
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- Poor enhancing inside ring: Swelling of intima
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- Can be used to assess treatment response
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- ## MR Findings
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- ### T1WI
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- **Wall thickening: Aorta****and****branches**
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- Wall edema can be seen in STIR images
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- ### T1WI C+
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- **Enhancement of thickened vessel wall**
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- ### MRA
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- Focal/diffuse narrowing of aorta and branches
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- Aortic dilatation (ascending > descending)
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- Stenosis > occlusion
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- Aortic regurgitation, dissection, aneurysm
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- Disease activity
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- Active disease: Wall edema and delayed enhancement can be seen
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- Poor and inconsistent correlation with disease activity
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- Development of new lesion in previously unaffected vascular territory is evidence of active disease/progression
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- Evaluate for new lesions in other territories
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- ## Ultrasonographic Findings
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- ### Grayscale ultrasound
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- Vascular wall thickening, dilation, stenosis
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- Diffuse arterial wall thickening: Macaroni sign
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- Carotid intima-media thickness > 1 mm is considered to be inflammatory vascular disease
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- ### Color Doppler
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- Luminal stenosis, change in blood flow patterns
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- 81% sensitivity and 100% specificity for diagnosing TA
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- ## Angiographic Findings
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- Early: Aortic wall thickening, rarely, stenosis
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- Late: Stenosis, occlusion, aneurysm; 4 types
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- I: Aortic arch branches
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- II: Thoracic aorta (a: Ascending, B: Descending) and branch vessels
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- III: Descending thoracic and abdominal aorta ± renal arteries; may have atypical coarctation
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- IV: Abdominal aortic ± renal arteries
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- V: Entire aorta and its branches
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- ## Nuclear Medicine Findings
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- ### PET
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- FDG uptake; ranges from low grade to intense
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- Treatment monitoring: Multiple qualitative and quantitative methods
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- ## Imaging Recommendations
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- ### Protocol advice
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- Multiplanar reconstructions for stenosis
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# DIFFERENTIAL DIAGNOSIS
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- [Giant Cell Arteritis](/document/giant-cell-arteritis/208eca17-81b8-448c-b8be-80e274dccc42)
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- Affects large vessels in older patients (> 50 years)
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- [Vasculitis Mimics](/document/coarctation-of-aorta/c0b23d8c-05e3-4373-b5d9-2de1590414a7)
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- Infections (tuberculosis, syphilis, HIV, bacterial)
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- Atherosclerosis; thromboembolism
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- Genetic disorders: Marfan, Ehlers-Danlos IV, Loeys-Dietz, Grange
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- Congenital: Coarctation, Turner syndrome, Williams syndrome
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- Unknown etiology: Fibromuscular dysplasia, segmental arterial mediolysis
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- [Behçet Disease](/document/behcet-syndrome/b08f488b-dd30-425e-9e5f-243c5feb477f)
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- Large vessel vasculitis seen in 30% of these patients
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- Proximal PA aneurysms are common
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- ## Other Causes of Aortitis
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- Ankylosing spondylitis, rheumatoid arthritis, Cogan syndrome, relapsing polychondritis, IgG4-related disease
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Autoimmune etiology is suspected
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- Infectious triggers suspected: Mycobacterium tuberculosis
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- Suspected association with active or latent TB
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- ### Genetics
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- Specific types of human leukocyte antigen are common
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- Strong association with HLA-B52
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- ## Gross Pathologic & Surgical Features
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- Wall thickening of large vessels
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- ## Microscopic Features
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- Granulomatous inflammation of arterial wall
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- Intimal proliferation; fibrosis of media and adventitia
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- **Early or prepulseless phase**
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- Low-grade fever, malaise, weight loss, fatigue
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- **Vascular inflammatory phase**
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- Vascular insufficiency
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- Symptoms are minimized by collateral formation
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- **Late quiescent occlusive or pulseless phase**
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- Diminished/absent pulses, vascular bruits, claudication
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- Blood pressure discrepancies in upper extremity
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- Subclavian steal syndrome
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- Hypertension, aortic regurgitation
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- Neurologic symptoms: Headache, dizziness, seizures, stroke
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- Ocular symptoms (ranging 8-68%): Hypertensive retinopathy or Takayasu retinopathy (due to hypoperfusion)
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- Triphasic pattern is seen in minority of patients
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- Disease is usually recurrent; phases may coexist
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- Interval between early and late phases is variable
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- **Cardiac involvement**
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- Myocardial ischemia: 84% of asymptomatic patients
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- Can cause angina, myocardial infraction, or sudden cardiac death
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- Coronary involvement is independent predictor of poor long-term outcomes
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- Type 1 (most common): Stenosis or occlusion of coronary ostia and proximal coronary artery
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- Type 2: Diffuse or focal coronary arteritis (skip lesions)
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- Type 3: Coronary aneurysms
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- ### Other signs/symptoms
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- Pulmonary hypertension when PA is involved
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- Increases risk of early mortality
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- ## Demographics
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- ### Age
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- Most common in 2nd and 3rd decades of life
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- ### Sex
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- F:M = 8:1
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- ### Epidemiology
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- Most common in Asia
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- Affects 6 out of 1,000 persons worldwide
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- Annual incidence in USA: 2-3
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- ## Natural History & Prognosis
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- Congestive heart failure is most common cause of death
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- Hypertension is poor prognostic factor
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- ## Treatment
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- Corticosteroids are 1st-line treatment; cyclophosphamide and methotrexate are 2nd line
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- Angioplasty, surgical bypass, or stent placement for stenosis and occlusion
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37263d6e-e027-4379-8e09-1e15b44df3b6
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## References
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# Selected References
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1. [Somashekar A et al: Updates in the diagnosis and management of Takayasu's arteritis. Postgrad Med. 135(sup1):14-21, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36588528%5Bpmid%5D)
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1. [Grayson PC et al: 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis. Arthritis Rheumatol. 74(12):1872-80, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36349501%5Bpmid%5D)
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1. [Jia S et al: Application progress of multiple imaging modalities in Takayasu arteritis. Int J Cardiovasc Imaging. 37(12):3591-601, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34287748%5Bpmid%5D)
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1. [Chatterjee S et al: Clinical diagnosis and management of large vessel vasculitis: Takayasu arteritis. Curr Cardiol Rep. 16(7):499, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24893936%5Bpmid%5D)
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1. [Khandelwal N et al: Multidetector CT angiography in Takayasu arteritis. Eur J Radiol. 77(2):369-74, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=19720487%5Bpmid%5D)
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1. [Restrepo CS et al: Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta. Radiographics. 31(2):435-51, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21415189%5Bpmid%5D)
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1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=18292120%5Bpmid%5D)
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1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=15855090%5Bpmid%5D)
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1. [Tso E et al: Takayasu arteritis: utility and limitations of magnetic resonance imaging in diagnosis and treatment. Arthritis Rheum. 46(6):1634-42, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12115196%5Bpmid%5D)
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## Tables
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# Classification Criteria for Takayasu Arteritis: 2022 ACR/EULAR
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| --- | --- |
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| Absolute Requirements |
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| Age ≤ 60 | |
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| Evidence of vasculitis on imaging | |
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| Additional Clinical Criteria |
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| Female sex | +1 |
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| Angina or ischemic cardiac pain | +2 |
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| Claudication | +2 |
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| Vascular bruit | +2 |
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| Diminished pulse in upper extremity | +2 |
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| Carotid artery abnormality | +2 |
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| Systolic BP difference in arms ≥ 20 mmHg | +1 |
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| Additional Imaging Criteria |
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| Number of affected artery territory | |
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| 1 arterial territory | +1 |
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| 2 arterial territories | +2 |
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| 3 or more arterial territories | +3 |
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| Symmetric involvement of paired arteries | +1 |
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| Abdominal aorta involvement with renal or mesenteric involvement | +3 |
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| Score of ≥ 5 points = Takayasu arteritis | |
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## Anatomy
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### Cervical Carotid Arteries
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Brain/ANATOMY:6eafa14e-2538-41b5-850c-29c41f38b970
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### Aortic Arch and Great Vessels
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Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
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### Pulmonary Vessels
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Chest/ANATOMY:551f4b47-fac1-44f9-b800-09ce766fedd9
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### Vessels, Lymphatic System, and Nerves, Abdominal
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Gastrointestinal/ANATOMY:0c38fd49-88e7-4272-960f-b17a194ce0cc
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### Carotid Space
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Head and Neck/ANATOMY:627bdee1-4bde-46f2-b93d-958882586337
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### Aorta and Inferior Vena Cava
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Ultrasound/ANATOMY:eed39e4d-478b-45d3-9406-1eace6e9eef1
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### Abdominal Aorta and Visceral Vasculature Anatomy
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Vasculature/ANATOMY:341c0af5-bfe8-4755-a401-39b7b9f2d9b9
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## Cases
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- {'cases': [{'authors': [{'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}, {'key': '3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1', 'value': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI'}], 'caseVersionId': '9dddd688-4ce9-4c92-b4c8-ec6f1ee538b0', 'description': 'Oblique axial volume rendered CECT (#1) shows severe narrowing (arrow) of the right interlobar pulmonary artery. Oblique sagittal VR CECT (#2) demonstrates moderate narrowing (arrow) of the proximal left lower lobe pulmonary artery. An oblique coronal VR CECT (#3) demonstrates severe narrowing of the right upper lobe (black arrow) and the interlobar pulmonary arteries (white arrow).', 'history': 'Patient with history of chronic dyspnea.', 'imagePoolId': '6e8f4a0f-9d6a-453e-8bb2-0f32a2b053eb', 'name': 'Takayasu arteritis', 'teachingPoint': None, 'demographics': '46 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
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## Images
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### Selected Images
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*Axial CECT in a patient with Takayasu arteritis (TA) shows marked thickening of the wall of the ascending thoracic aorta <img src='img/arrows/WC.png'/>. The thoracic aorta and its branches, particularly the left subclavian artery, are the most commonly affected vessels in TA.*
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*Axial CECT in a patient with Takayasu arteritis (TA) shows marked thickening of the wall of the ascending thoracic aorta <img src='img/arrows/WC.png'/>. The thoracic aorta and its branches, particularly the left subclavian artery, are the most commonly affected vessels in TA.*
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*Axial T1 MR in a patient with TA demonstrates thickening of the wall of the ascending thoracic aorta <img src='img/arrows/WO.png'/> and the pulmonary trunk <img src='img/arrows/WC.png'/>. Vessel stenosis, occlusion, and aneurysm formation may complicate cases of TA.*
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*Axial fused PET/CT in a patient with active TA demonstrates intense FDG uptake within the mediastinum adjacent to the aortic arch <img src='img/arrows/WS.png'/> in a region of soft tissue attenuation that was present on the localization CT.*
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*Axial fused PET/CT in the same patient shows intense FDG uptake within the mediastinum adjacent to the ascending aorta and pulmonary arteries <img src='img/arrows/WC.png'/>. FDG uptake may be low grade to intense in TA, and PET/CT is an effective way of monitoring treatment response.*
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*Composite image with axial CECT shows wall thickening <img src='img/arrows/BS.png'/> of the proximal right brachiocephalic, left common carotid, and left subclavian arteries. There is more distal occlusion <img src='img/arrows/WC.png'/> of the left common carotid and left subclavian arteries. The left subclavian artery is the most common branch vessel affected in patients with TA.*
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*Sagittal oblique DSA in another patient with TA shows patency of the left common carotid artery with occlusion of the right brachiocephalic <img src='img/arrows/BO.png'/> and left subclavian arteries <img src='img/arrows/BS.png'/>.*
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*Axial CECT in a patient with TA shows soft tissue attenuation and no contrast opacification within the left subclavian artery <img src='img/arrows/WS.png'/>, consistent with occlusion.*
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*Sagittal oblique DSA in the same patient with TA shows occlusion of the left subclavian artery <img src='img/arrows/BS.png'/>. Note the common origin <img src='img/arrows/WO.png'/> of the right brachiocephalic and left common carotid arteries.*
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*Composite image with axial T1 MR (left) and DSA (right) shows aortic wall thickening <img src='img/arrows/WS.png'/> and a focal aneurysm <img src='img/arrows/WC.png'/> confirmed on subtraction aortic DSA <img src='img/arrows/BS.png'/>.*
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*Axial CECT in a patient with TA shows an aortic dissection with marked intramural hemorrhage surrounding the false lumen <img src='img/arrows/WS.png'/>. TA was confirmed on pathologic examination of the resected specimen.*
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### Additional Images
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*Axial T1 MR in a patient with TA shows abnormal high signal within the left common carotid artery <img src='img/arrows/BO.png'/>, consistent with occlusion.*
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*Axial T1 MR in the same patient shows an absence of flow within the left subclavian artery <img src='img/arrows/WS.png'/>, consistent with luminal occlusion.*
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