Giant Cell Arteritis

  • Autoimmune inflammation of medium-sized arteries, especially branches of external carotid artery
  • Affects those aged 60 years and older
  • May be severe form of polymyalgia rheumatica
  • Causes headache, joint and muscle aches, scalp tenderness, jaw claudication, fatigue, appetite loss
  • Elevated erythrocyte sedimentation rate and C-reactive protein
  • Chief concern is arteritic ischemic optic neuropathy causing irreversible blinding infarction of optic disc in one or both eyes
  • Sudden painless loss of vision in one eye
  • Similar process may affect other eye within hours to days
  • Visual loss often profound
  • Afferent pupil defect, and...
  • Swollen optic disc in affected eye(s) in 95% of cases
  • In at least 80% of cases, patient reports one or more systemic features present for weeks to months
  • Refer emergently to ophthalmologist any elderly patient with sudden visual loss, even if there are no systemic features of giant cell arteritis
  • Refer elderly patients for temporal artery biopsy if they have new head or neck manifestations that suggest giant cell arteritis and sedimentation rate and C-reactive protein are elevated
  • Patients with clinical evidence of optic disc infarction and clinical or serologic evidence to suggest giant cell arteritis will be treated immediately with intensive intravenous corticosteroid therapy
  • Intensive corticosteroid treatment may prevent further visual loss
  • Patients who have normal vision but systemic symptoms of polymyalgia rheumatic or giant cell arteritis will undergo testing of sedimentation rate and C-reactive protein, which will be elevated in at least 80% of cases with giant cell arteritis
  • To sustain diagnosis of giant cell arteritis, temporal artery biopsy must show thickening of media and endothelium, often with fragmentation of internal elastic lamina and sometimes with granulomas filled with Langerhans giant cells (granulomatous inflammation)
  • Properly performed and interpreted, temporal artery biopsy of one side has sensitivity of at least 93% and specificity of nearly 100% for giant cell arteritis
  • If biopsy of one temporal artery is negative, biospy of other temporal artery is indicated in cases of high clinical suspicion
  • Biopsy of second temporal artery increases sensitivity by about 3%
  • Negative biopsy excludes diagnosis of giant cell arteritis, and...
  • Positive biopsy mandates continuous corticosteroid treatment for at least 1 year after diagnosis