Extracranial giant cell arteritis: A narrative review

Neth J Med. 2016 Jun;74(5):182-92.


A systematic literature search was performed to summarise current knowledge on extracranial giant cell arteritis (GCA), i.e. large-artery involvement in patients with or without clinically apparent temporal arteritis (cranial GCA). Extracranial GCA is increasingly recognised, both in patients with cranial GCA and with solitary extracranial GCA, due to increased awareness among physicians and development of modern imaging modalities. The literature on the pathogenesis and histopathology of extracranial GCA is scarce. It is considered to be similar to cranial GCA. Patients with solitary extracranial GCA often present with non-specific signs and symptoms, although vascular manifestations, mostly secondary to stenosis, may occur. Due to the non-specific clinical presentation and low sensitivity of temporal artery biopsies, extracranial GCA is usually diagnosed by imaging. 18F-FDG-PET, MRI, CT angiography and ultrasound are used for this purpose. At present, the optimal diagnostic strategy is undetermined. The choice for a particular modality can be guided by the clinical scenario that raises suspicion of extracranial GCA, in addition to local availability and expertise. Extracranial complications in GCA consist of aortic aneurysm or dissection (mainly the ascending aorta), aortic arch syndrome, arm claudication and posterior stroke (although this is technically a cranial complication, it often results from stenosis of the vertebrobasilar arteries). Mortality is generally not increased in patients with GCA. Treatment of patients with solitary extracranial and those with extracranial and cranial GCA has been debated in the recent literature. In general, the same strategy is applied as in patients with temporal arteritis, although criteria regarding who to treat are unclear. Surgical procedures may be indicated, in which case optimal medical treatment prior to surgery is important.

Publication types

  • Review

MeSH terms

  • Aneurysm, Dissecting / etiology
  • Aortic Aneurysm / etiology
  • Aortic Arch Syndromes / etiology
  • Aortitis / complications
  • Aortitis / diagnostic imaging*
  • Aortitis / pathology
  • Aortitis / therapy
  • Axillary Artery / diagnostic imaging
  • Axillary Artery / pathology
  • Biopsy
  • Carotid Artery Diseases / complications
  • Carotid Artery Diseases / diagnostic imaging*
  • Carotid Artery Diseases / pathology
  • Carotid Artery Diseases / therapy
  • Computed Tomography Angiography
  • Femoral Artery / diagnostic imaging
  • Femoral Artery / pathology
  • Fluorodeoxyglucose F18
  • Giant Cell Arteritis / complications
  • Giant Cell Arteritis / diagnostic imaging*
  • Giant Cell Arteritis / pathology
  • Giant Cell Arteritis / therapy
  • Glucocorticoids / therapeutic use
  • Humans
  • Iliac Artery / diagnostic imaging
  • Iliac Artery / pathology
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Angiography
  • Mesenteric Arteries / diagnostic imaging
  • Mesenteric Arteries / pathology
  • Positron Emission Tomography Computed Tomography
  • Prognosis
  • Radiopharmaceuticals
  • Renal Artery / diagnostic imaging
  • Renal Artery / pathology
  • Subclavian Artery / diagnostic imaging
  • Subclavian Artery / pathology
  • Temporal Arteries / diagnostic imaging
  • Temporal Arteries / pathology
  • Vascular Surgical Procedures


  • Glucocorticoids
  • Immunosuppressive Agents
  • Radiopharmaceuticals
  • Fluorodeoxyglucose F18