NEUROIMAGING

Ultrasound in the diagnosis and management of giant cell arteritis (GCA)

David Goldemund M.D.
Updated on 22/10/2024, published on 22/10/2024
  • in the acute GCA, ultrasound shows a non-compressible, hypoechoic wall thickening of the temporal and other arteries
  • reliability is excellent and comparable to biopsy
  • ultrasound should be used as a first-line diagnostic test for patients with suspected GCA; rapid examination and initiation of therapy reduce the risk of permanent vision loss

Introduction

  • permanent vision loss due to anterior ischemic optic neuropathy is a serious complication of GCA, occurring mainly in untreated patients ⇒ prompt diagnosis and treatment are thus crucial
  • glucocorticoid therapy should be started immediately
  • ultrasound (US) is a safe, inexpensive, and readily available diagnostic tool with characteristic findings in both GCA and Takayasu arteritis
  • immediate US examination has 2 advantages:
    • physicians performing both clinical and ultrasound exams can provide instantaneous diagnosis and treatment, reducing the risk of delayed treatment or unnecessary glucocorticoid use.
    • rapid ultrasound visualizes pathologic changes before treatment alters their appearance (the visibility of the halo sign decreases rapidly after 2-3 days of treatment) (Schmidt, 2018) 
    • delayed US may yield false-negative results
  • temporal artery biopsy may remain positive for weeks

Technical requirements

  • imaging should be performed by specialists with appropriate equipment (> 300 US examinations are required).
  • modern high-resolution linear probes with Doppler mode should be used, especially for temporal artery studies
  • US resolution increases with higher frequencies, and tissue penetration increases with lower frequencies
  • probes with ≥ 15MHz frequency should be used to detect minor wall thickening.
    • probes with frequencies >20MHz are increasingly available, and such probes allow visualization of the normal intima-media complex of the temporal arteries

Pathologic US findings correspondig with GCA

  • a normal arterial intima-media complex appears on ultrasound as a homogeneous, hypo- or anechoic structure bounded by two parallel hyperechoic margins
  • the following pathological features may be seen on the US in patients with GCA:
    • hypoechoic wall thickening (halo sign)  Halo sign in giant cell arteritis (Schmidt, 2018) 
      • high-frequency probe is required (≥ 15-20 MHz)
      • edema/fluid is black
      • the cut-off for the common superficial temporal artery 0.42 mm, frontal branch 0.34 mm, parietal branch 0.29 mm, and axillary artery 1.0 mm  (Schäfer, 2017)
    • non-compressible arteries  Normal compressibility of healthy STA
      • a positive compression sign is defined as the visibility of the TA during transducer-imposed compression of the artery  (Aschwanden, 2013)
    • lumen stenosis (turbulent flow, increased velocity) or occlusion (absence of Doppler signal)
      • stenosis should be considered only to assess the severity of damage and not to confirm the diagnosis of GCA
Superficial temporal artery on the ultrasound - above the enlarged wall in the acute stage, below normalization after corticotherapy [Suelves, 2010]

Which arteries are examined

  • temporal and axillary arteries (routinely examined in suspected GCA)
    • routine examination of the axillary arteries is essential in suspected GCA cases, as temporal arteries may be spared in 40% of cases
    • superficial temporal arteries should be scanned in both longitudinal and transverse planes + frontal and parietal branches
    • axillary arteries are commonly involved in younger, predominantly female patients
  • occipital and facial arteries
    • facial arteries wind around the body of the mandible; occipital arteries are located behind the ear
    • involved in 30-40% of GCA patients
    • facial arteritis causes more often jaw claudication and blindness
  • carotid arteries – CCA is more often involved than ICA and ECA; arteriosclerosis and vasculitis can be difficult to differentiate
    • arteriosclerosis is characterized by heterogeneous (sometimes hyperechoic), irregular, and eccentric changes int he vessel wall  Vasculitis vs. atherosclerosis on the ultrasound
  • subclavian and vertebral arteries
    • reversed flow in the VA indicates high-grade subclavian stenosis (subclavian steal phenomenon)
    • subclavian arteries are insonated from above and below the clavicle
  • aorta
    • the lungs interfere with examination of the thoracic aorta
    • only part of the ascending aorta and the arch can be examined with low-frequency probes
    • transesophageal echocardiography providing high-resolution images of the thoracic aorta is not routinely used
    • CTA or MRA are preferred in this indication  Concentric thickening of the aortic wall on CTA
  • axillary artery imaging is typically performed in the axilla
  • the patient’s arm is abducted and externally rotated to expose the axilla
  • for a transverse view, the probe is placed perpendicular to the artery, visualizing a cross-section of the vessel
  • for a longitudinal view, the probe is aligned parallel to the artery, providing a longitudinal view of the vessel
  • the axillary artery should be differentiated from:
    • the axillary vein (which is typically more compressible and larger)
    • lymph nodes
    • brachial plexus
    • muscles (such as the pectoralis major and minor)

Ultrasound vs. other methods

  • ultrasound – when performed by experienced staff, along with clinical examination, can confirm or exclude a suspected diagnosis of GCA in most patients
  • US vs. biopsy
    • temporal artery biopsy (TAB) may be necessary when US results are unclear, especially in those on long-term glucocorticoid treatment
    • US-guided TAB does not significantly increase sensitivity over standard TAB
    • US is faster and less expensive than TAB, reducing costs and waiting time
    • TAB is less sensitive because it covers a limited area, whereas the US covers more regions in generalized disease
  • US vs. MRI/CT/PET  Probable GCA on the right image (CTA)   
    • MRI, CT, and PET are better for visualizing large vessels such as the thoracic aorta but they are expensive and involve radiation ⇒ US is a better choice for most patients unless there is suspicion of aortic involvement

 

Ultrasound disease monitoring

  • with treatment, the halo becomes brighter, and its diameter decreases
    • it may resolve within days to months after treatment is initiated (Hauhenstein,2012)
    • residual wall thickening may remain visible for years, particularly in patients with temporal artery halo or occlusion
    • in extracranial arteries (CCA, axillary artery), wall thickening usually remains visible for months or years
  • ultrasound monitoring might become more important in the future with treatments involving IL-6
    • inhibition may reduce the usefulness of CRP and ESR measurements
  • wall thickness can be checked twice a year;  increasing thickness suggests undertreatment
  • newer techniques may visualize other markers of disease activity, such as neovascularization on contrast-enhanced US

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Ultrasound in the diagnosis and management of giant cell arteritis
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