Takayasu Arteritis

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 onsetDevelopment of symptoms or findings related to Takayasu arteritis at age <40 years
    Claudication of extremitiesDevelopment and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities
    Decreased brachial artery pulseDecreased pulsation of one or both brachial arteries
    BP difference >10mmHgDifference of >10mmHg in systolic blood pressure between arms 
    Bruit over subclavian arteries or aortaBruit 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 et al)
    • 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
    • Erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP)  – elevated
    • 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

Clinical Background

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

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

Initial evaluation for suspected  vasculitis

   
C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

Initial evaluation for suspected vasculitis

   
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

Initial evaluation for suspected ANCA-associated 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

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

May help in ruling out infectious process

   
Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

Screen for hematuria, proteinuria, and RBC casts