Takayasu Arteritis

Indications for Testing

Clinical symptoms suggestive of Takayasu arteritis
- Asymmetric blood pressure
- Decrease of brachial artery pulse
- Bruit over subclavian arteries or aorta
- Claudication of extremities
- Blood pressure difference between arms

Criteria for Diagnosis

American College of Rheumatology criteria for the classification of Takayasu arteritis (1990)

Criteria	Definition
Age at disease onset	Symptoms or findings related to Takayasu arteritis before age 40 years
Claudication of extremities	Development/worsening of fatigue and discomfort in muscles of ≥1 extremity while in use, particularly upper extremities
Decreased brachial artery pulse	Decreased pulse in 1 or both brachial arteries
BP difference >10mmHg	Difference in systolic BP of >10 mmHg between arms
Bruit over subclavian arteries or aorta	Bruit over 1 or both subclavian arteries or abdominal aorta audible on auscultation
Arteriogram abnormality	Arteriographic narrowing or occlusion of entire aorta, its primary branches, or large arteries in proximal upper or lower extremities, not as a result of arteriosclerosis, fibromuscular dysplasia, or similar causes – changes typically focal or segmental
Source: American College of Rheumatology BP=blood pressure For purposes of classification, a patient will be considered to have Takayasu's arteritis if ≥3 of these 6 criteria are present The presence of ≥3 criteria yields a sensitivity of 90.5% and a specificity of 97.8%

Ishikawa's criteria for Takayasu arteritis (modified according to Sharma, 1995)
- Proposed modifications to criteria include
  - Removal of age criterion of <40 years
  - Inclusion of characteristic signs and symptoms as a major criterion
  - Removal of age in defining abdominal aortic lesion
  - Addition of coronary artery lesion in absence of risk factors

Laboratory Testing

Nonspecific testing – helpful in excluding other diagnoses or identifying organ dysfunction
- Urinalysis – negative for hematuria
- CBC – may show elevated white blood cell (WBC) count
- C-reactive protein (CRP)
  - Preferred test to detect inflammatory processes (Choosing Wisely, American Society for Clinical Pathology, 2016)
  - Usually elevated
  - If CRP not available, order erythrocyte sedimentation rate (ESR)
- Antineutrophil cytoplasmic antibodies (ANCA) – rule out ANCA-associated vasculitis

Histology

Inflammation, granulomas, and giant cells in large artery walls
Often indistinguishable from giant cell arteritis

Imaging Studies

Magnetic resonance angiography (MRA)/computed tomography (CT) angiography – narrowings, occlusions, or aneurysms of the aorta and its branches
- Ultrasound (US) is not as well established as a diagnostic tool

Differential Diagnosis

Mycotic aneurysm
Atherosclerosis
Congenital – aortic coarctation
Hereditary disorders
- Ehlers-Danlos syndrome
- Marfan syndrome
- Loeys-Dietz syndrome
Fibromuscular dysplasia
Postradiation syndrome
Chronic aortic aneurysm
Chronic infection
- Mycobacterium tuberculosis
- Treponema pallidum

Vasculitis in Adults Testing Algorithm

Read more about Vasculitis in Adults Testing Algorithm

Vasculitis in Children Testing Algorithm

Read more about Vasculitis in Children Testing Algorithm

Takayasu arteritis is a form of granulomatous arteritis that primarily affects the aorta and its major branches. It is most prevalent in adolescent girls and young women and is characterized by ocular disturbances and marked weakening of the pulses in the upper extremities. It is categorized as a large-vessel vasculitis (Jennette, 2013).

Epidemiology

Incidence – rare
Age – 20s (mean)
Sex – F>M, 29:1 in U.S.
Ethnicity – predominantly Asian

Pathophysiology

Large-vessel vasculitis characterized by adventitial thickening and cellular infiltration of tunica media with local destruction of vascular smooth muscle cells and elastin
- Strong predilection for aorta, its branches, and pulmonary arteries
Pathogenic T-lymphocyte response

Clinical Presentation

Constitutional – fever, fatigue, malaise, weight loss
Cardiovascular – hypertension, asymmetric blood pressure between limbs, diminished pulses, bruits (carotid most common)
Musculoskeletal – arthralgias, myalgias, limb claudication
Neurologic – syncope, lightheadedness, headache, visual disturbances

Tests generally appear in the order most useful for common clinical situations. Click on number for test-specific information in the ARUP Laboratory Test Directory.

C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

Recommended Use

Preferred test to detect acute phase inflammation (eg, autoimmune diseases, connective tissue disease, rheumatoid arthritis, infection, or sepsis)

Anti-Neutrophil Cytoplasmic Antibody with Reflex to Titer and MPO/PR-3 Antibodies 2002068
Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay

Recommended Use

Preferred reflex panel for managing patients with known diagnosis of vasculitis; may assist in evaluating suspected vasculitis

For the workup of suspected vasculitis, preferred panel is ANCA-associated vasculitis profile (ANCA/MPO/PR-3) with reflex to ANCA titer

Panel includes ANCA (IgG), myeloperoxidase antibody, and serine protease 3 antibody

Reflex pattern – if screen is positive, then titer and MPO/PR-3 antibodies testing will be added to aid in antibody determination

Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

Recommended Use

Screen for various metabolic and kidney disorders

CBC with Platelet Count and Automated Differential 0040003
Method: Automated Cell Count/Differential

Recommended Use

May help in ruling out infectious process

Guidelines

Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990; 33(8): 1129-34. PubMed

Choosing Wisely. An initiative of the ABIM Foundation. [Accessed: Sep 2017]

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65(1): 1-11. PubMed

General References

Alibaz-Oner F, Aydin SZ, Direskeneli H. Advances in the diagnosis, assessment and outcome of Takayasu's arteritis. Clin Rheumatol. 2013; 32(5): 541-6. PubMed

Cakar N, Yalcinkaya F, Duzova A, Caliskan S, Sirin A, Oner A, Baskin E, Bek K, Soylu A, Fitoz S, Bayazit AK, Bircan Z, Ozen S, Uncu N, Ekim M. Takayasu arteritis in children. J Rheumatol. 2008; 35(5): 913-9. PubMed

Chatterjee S, Flamm SD, Tan CD, Rodriguez R. Clinical diagnosis and management of large vessel vasculitis: Takayasu arteritis. Curr Cardiol Rep. 2014; 16(7): 499. PubMed

de Souza AW, de Carvalho JF. Diagnostic and classification criteria of Takayasu arteritis. J Autoimmun. 2014; 48-49: 79-83. PubMed

Seko Y. Giant cell and Takayasu arteritis. Curr Opin Rheumatol. 2007; 19(1): 39-43. PubMed

Sharma BK, Siveski-Iliskovic N, Singal PK. Takayasu arteritis may be underdiagnosed in North America. Can J Cardiol. 1995; 11(4): 311-6. PubMed