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	Development of symptoms or findings related to Takayasu arteritis at age <40 years
Claudication of extremities	Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities
Decreased brachial artery pulse	Decreased pulsation of one or both brachial arteries
BP difference >10mmHg	Difference of >10mmHg in systolic blood pressure between arms
Bruit over subclavian arteries or aorta	Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta
Arteriogram abnormality	Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibro-muscular dysplasia, or similar causes – changes usually focal or segmental
Source: American College of Rheumatology For purposes of classification, a patient shall be said 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%. BP = blood pressure (systolic) difference between arms

Ishikawa's criteria for Takayasu arteritis (modified according to Sharma, 1995)
- Proposed modifications to criteria include
  - Removal of criterion of age <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 WBC
- C-reactive protein (CRP)
  - Preferred test to detect inflammatory processes (Choosing Wisely: 5 Things Physicians and Patients Should Question, 2015; American Society for Clinical Pathology)
  - Usually elevated
  - If CRP not available, order erythrocyte sedimentation rate (ESR)
- ANCA – rule out ANCA-associated vasculitis

Histology

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

Imaging Studies

MRA/CT angiography – narrowings, occlusions, or aneurysms of the aorta and its branches
- US not as well established as 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 affects primarily 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. (Chapel Hill 2012)

Epidemiology

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

Pathophysiology

Large-vessel vasculitis characterized by adventitial thickening and cellular infiltration of the 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 a known diagnosis of vasculitis; may be assistive in evaluating suspected vasculitis

For the workup of suspected vasculitis, the 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

Limitations

Not specific for Takayasu arteritis

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

Recommended Use

May help in ruling out infectious process

Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

Recommended Use

Screen for hematuria, proteinuria, and RBC casts

Guidelines

American Society for Clinical Pathology. Choosing Wisely - Five Things Physicians and Patients Should Question. An initiative of the ABIM Foundation. [Last revision Feb 2015; Accessed: Jan 2016]

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

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