Takayasu Arteritis

Indications for Testing

Clinical symptoms suggestive of Takayasu arteritis

Criteria for Diagnosis

American College of Rheumatology and Ishikawa's criteria for Takayasu arteritis

Laboratory Testing

Non-specific testing (not helpful in diagnosis)
- Urinalysis – negative for hematuria
- CBC – may show elevated WBC
- ESR/CRP – elevated
- ANCA – rule out other vasculitides that are typically ANCA-positive

Histology

Inflammation, granulomas and giant cells in large artery walls; pathology is indistinguishable from giant cell arteritis

Imaging Studies

MRI/US/CT angiography – narrowings, occlusions or aneurysms of the aorta and its branches

Treatment

Steroids are first-line therapy
May require addition of other immunosuppressives

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

Takayasu arteritis is a form of granulomatous arteritis that affects large-sized arteries (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; for this reason, it is also referred to as pulseless disease.

Epidemiology

Incidence – 2-3/100,000 in the U.S.
Age – mean 20s
Sex – F>M (29:1 in the U.S.)
Ethnicity – predominantly Asian

Pathophysiology

Systemic 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

Treatment

Corticosteroids – first line therapy
Immunosuppressants
Antihypertensives
Vascular interventions (eg, bypass procedure)

Tests generally appear in the order most useful for common clinical situations

Test name: CBC with Platelet Count & Automated Differential

ARUP #: 0040003

Methodology: Automated Cell Count with Flow Cell Differential

Use:

Differentiate between infectious and non-infectious process

Test name: Urinalysis, Complete

ARUP #: 0020350

Methodology: Reflective Photometry/Microscopic by Yellow IRIS

Use:

Evaluate for presence of hematuria

Test name: Sedimentation Rate, Westergren (ESR)

ARUP #: 0040325

Methodology: Westergren

Use:

Assess for inflammation

Test name: C-Reactive Protein

ARUP #: 0050180

Methodology: Quantitative Immunoturbidimetric

Use:

Assess for inflammation

Test name: Anti-Neutrophil Cytoplasmic Antibody with Reflex to Titer and MPO/PR-3 Antibodies

ARUP #: 2002068

Methodology: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multi-Analyte Fluorescent Detection

Use:

Rule out ANCA-associated systemic vasculitis

If screen is positive, titer and MPO/PR-3 antibodies testing will be added to aid in antibody determination

Limitations:

Not specific for Takayasu arteritis

General

Comprehensive Review: June 2011
Last Update: February 2012

Giant Cell Arteritis

Mycobacterium tuberculosis

Treponema pallidum

Vasculitis