Takayasu Arteritis

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).

Quick Answers for Clinicians

Which testing algorithms are related to this topic?
Vasculitis in Adults Testing Algorithm
Vasculitis in Children Testing Algorithm

Diagnosis

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

Note: 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%.

BP, blood pressure

Source: American College of Rheumatology

  • 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

Background

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

ARUP Lab Tests

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

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/PR3) with reflex to ANCA titer

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

Reflex pattern: if screen is positive, then titer and MPO/PR3 antibodies testing will be added to aid in antibody determination

Screen for various metabolic and kidney disorders

May help in ruling out infectious process

Related Tests

Nonspecific test used to detect inflammation associated with infections, cancers, and autoimmune diseases

Medical Experts

Contributor

Genzen

Jonathan R. Genzen, MD, PhD

Jonathan R. Genzen, MD, PhD

Associate Professor of Clinical Pathology, University of Utah

Chief Operations Officer, Medical Director of Automated Core Laboratory and Farmington Health Center Clinical Laboratory, at ARUP Laboratories

Contributor

References

Additional Resources

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