Giant Cell Arteritis (Temporal Arteritis) Guidelines

Updated: Jul 07, 2022
  • Author: Mythili Seetharaman, MD; Chief Editor: Herbert S Diamond, MD  more...
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Guidelines

Guidelines Summary

The following organizations have published guidelines for the diagnosis and treatment of giant cell arteritis (GCA):

  • American College of Rheumatology/Vasculitis Foundation (ACR/VF) [167]
  • British Society for Rheumatology (BSR) [149]
  • European League Against Rheumatism (EULAR) [168]

Diagnosis

All the guidelines agree that a diagnosis of GCA should be confirmed either by temporal artery ultrasound (US) or temporal artery biopsy. [149, 167, 168]

In 2018, EULAR released an update to its imaging guidelines that includes the following key recommendations [116] :

  • In patients with suspected GCA, early imaging is recommended to complement the clinical diagnostic criteria, assuming high expertise and prompt availability of the imaging technique. Imaging should not delay initiation of treatment.
  • In patients in whom there is a high clinical suspicion of GCA and a positive imaging study, the diagnosis of GCA may be made without biopsy or further imaging. In patients with a low clinical probability and a negative imaging result, the diagnosis of GCA can be considered unlikely. In all other situations, additional efforts towards a diagnosis are necessary.
  • Temporal artery US, with or without axillary artery US, is recommended as the first imaging modality in patients with suspected GCA with predominantly cranial manifestations (eg, headache, visual symptoms, jaw claudication, temporal artery swelling and/or tenderness). A non-compressible ‘halo’ sign is the US finding most suggestive of GCA.
  • If US is not available or the results are inconclusive, high-resolution magnetic resonance imaging (MRI) of cranial arteries to investigate mural inflammation may be used as an alternative for diagnosis of GCA.
  • Computed tomography (CT) and positron emission tomography (PET) are not recommended for the assessment of cranial artery inflammation.
  • US, PET, MRI, and/or CT may be used for detection of mural inflammation and/or luminal changes in extracranial arteries to support the diagnosis of large-vessel GCA. US is of limited value for assessment of aortitis.
  • Conventional angiography is not recommended for the diagnosis of GCA as it has been superseded by the previously mentioned imaging modalities.
  • In patients with GCA in whom a flare is suspected, imaging might be helpful to confirm or exclude it. Imaging is not routinely recommended for patients in clinical and biochemical remission.
  • MRA, CTA, and/or US may be used for long-term monitoring of structural damage in patients with GCA, particularly to detect stenosis, occlusion, dilatation, and/or aneurysms. The frequency of screening as well as the imaging method applied should be decided on an individual basis.
  • Imaging examination should be done by a trained specialist using appropriate equipment, operational procedures, and settings. The reliability of imaging, which has often been a concern, can be improved by specific training.

Medical treatment

High-dose glucocorticoid (GC) therapy (40–60 mg/day prednisone equivalent) is recommended by all three guidelines as the initial treatment for GCA. [149, 167, 168]

For patients with threatened visual loss, both the ACR/VF and BSR guidelines recommend intravenous GC therapy over high-dose oral GC therapy. [149, 167] The ACR/VF guidelines conditionally recommend the combination of high-dose GC therapy and tocilizumab over GC therapy alone. [167] The BSR and EULAR guidelines recommend considering the use of methotrexate or tocilizumab during the tapering of GC therapy for patients with high risk of GC toxicity or for patients whose GCA relapses. [149, 168]  

ACR/VF and BSR guidelines recommend against the use of statins for the treatment of GCA. [149, 167, 168]

Once the disease is in remission, EULAR guidelines recommend tapering the GC dose to a target dose of 15–20 mg/day within 2–3 months and after 1 year to ≤5 mg/day. [168]

Surgical treatment

The ACR/VF guidelines include the following recommendations for surgical treatment of GCA [167] :

  • The type and timing of surgical intervention should be a collaborative decision between the vascular surgeon and rheumatologist
  • Escalation of immunosuppressive therapy is conditionally recommended over surgical intervention for severe GCA and worsening signs of limb/organ ischemia
  • For patients undergoing vascular surgery, high-dose GC therapy should continue during the periprocedural period, if the patient has active disease.

EULAR recommends that any elective surgical interventions or reconstructive surgery be performed when the patient is in a stable remission.  Arterial vessel dissection or critical vascular ischemia should receive urgent referral to a vascular surgeon. [168]