Epidemiology


Etiology


  • Association with polymyalgia rheumatica (PMR): up to 50% of patients with giant cell arteritis also have PMR.

Pathophysiology


  • Monocytes differentiate into macrophages and giant cells, which produce cytokines (e.g., IL-6, TNF-α) that augment the inflammatory response → focal granulomatous inflammation
  • Most commonly involves external carotid artery branches (especially temporal artery), as well as the aorta and vertebral arteries

Clinical features


  • Cranial giant cell arteritis: involves the extracranial branches of the common carotid, internal carotid, and external carotid arteries (the temporal artery is the most commonly affected vessel)
    • New-onset unilateral (or bilateral) headache
      • Can be pulse-synchronous, throbbing, dull
      • Typically located over the temples
    • Hardened and tender temporal artery Pasted image 20240403193138.png
    • Jaw claudication: jaw pain when chewing
    • Vision loss: due to inflammation and occlusion of the ophthalmic artery and its branches
      • Scintillating scotoma
      • Amaurosis fugax or permanent loss of vision due to anterior ischemic optic neuropathy (AION)
  • Symptoms of polymyalgia rheumatica, e.g. muscle pain primarily affecting shoulders, neck, and pelvic girdle

Diagnostics


  • Elevated erythrocyte sedimentation rate & C-reactive protein (best initial test)
    • Highly sensitive and almost always significantly elevated in GCA.
  • Temporal artery biopsy: intimal thickening, elastic lamina fragmentation, multinucleated giant cells

Treatment