Takayasu Arteritis

Diagnosis

Indications for Testing

  • Clinical symptoms suggestive of Takayasu arteritis

Criteria for Diagnosis

Laboratory Testing

  • Nonspecific 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 indistinguishable from giant cell arteritis

Imaging Studies

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

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

Clinical Background

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 U.S.
  • Age – mean 20s
  • Sex – F>M, 29:1 in 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)

Indications for Laboratory Testing

  • 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
Test Name and Number Recommended Use Limitations Follow Up
CBC with Platelet Count and Automated Differential 0040003
Method: Automated Cell Count/Differential

Differentiate between infectious and noninfectious process

   
Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

Evaluate for presence of hematuria

   
Sedimentation Rate, Westergren (ESR) 0040325
Method: Visual Identification

Assess for inflammation

   
C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

Assess for inflammation

   
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

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

Not specific for Takayasu arteritis