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A 75 year old woman with 4 episodes of "absence of vision"
Digital Journal of Ophthalmology 2002
Volume 8, Number 5
June 1, 2002
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Joseph Rizzo, M.D. | Harvard Medical School, Boston MA
Nicoletta Fynn-Thompson, M.D. | Massachusetts Eye and Ear Infirmary, Boston MA
Diagnosis and Discussion
Giant cell arteritis

Giant cell arteritis (GCA) is a systemic disease that affects patients over the age of 55 years. Caucasian patients are most commonly affected; it is very uncommon in patients of African descent. Giant cell arteritis predominantly involves medium sized blood vessels of the cranium, heart, and eye. The pattern of arterial involvement is directly proportional to the amount of elastic tissue found in the media and adventitia. The posterior ciliary artery and temporal artery are rich in elastic tissue, hence their high incidence of involvement. Interestingly, the cranial vessels are protected FROM this inflammatory disease once they penetrate the dura mater.

Typical complications of untreated disease include blindness, stroke, and death secondary to ischemia. Ocular manifestations include anterior ischemic optic neuropathy (AION), central retinal artery occlusion, and (less commonly) cranial nerve palsies. Most commonly patients present with severe and permanent visual loss in one eye. Transient visual symptoms may occur in advance or permanent blindness. The second eye usually becomes blind in days to weeks if the disease is not treated appropriately.

A recent study by Hayreh et al revealed that jaw claudication, neck pain, erythrocyte sedimentation rate (ESR) of 47-107mm/hr, and C-reactive protein (CRP) above 2.45mg/dl are highly correlated with a positive temporal artery biopsy. A CRP is the most sensitive serologic test - it is 100% sensitive, compared to a sensitivity of 92% for the ESR. They also SHOW that ESR and CRP together provide the best specificity (97% specific) for the serologic diagnosis. However, the definitive diagnosis is achieved by a positive temporal artery biopsy.

A positive temporal artery biopsy shows evidence of chronic vasculitis characterized by granulomatous or epitheliod inflammation. Giant cells may or may not be seen. There is focal destruction of the internal elastical lamina, but this is a nonspecific sign that is seen as the result of aging alone. Temporal artery biopsy is 100% specific and 65-95% sensitive for the GCA. To address the variability in sensitivity, recent literature has discussed the diagnostic yield of bilateral biopsies. Three recent publications , , found the diagnosis on the two sides to be discordant in 1-5% of retrospective case reviews.

Occult giant cell arteritis is ocular involvement by GCA without any systemic symptoms. A recent prospective study by Hayreh et al showed the incidence to be 21.2%, with no influence by age or sex of the individual. The majority of ocular ischemia was AION (94.4%), and less commonly, central retinal artery occlusion (5.6%). The values of ESR and CRP are generally lower in this patient population.

Treatment is with oral prednisolone or high-dose intravenous methylprednisolone, dependent on the severity or extent of involvement. Side effects of prolonged steroid use include osteoporosis, increase in total body fat, and increase in glucose levels. We recommend a starting dose of at least 80mg per day of prednisone. Higher doses (1gram of methylprednisolone per day) can be used if the threat of ischemia is more severe.