Giant Cell Arteritis

Andrew G. Lee, MD

Introduction

Giant cell arteritis is a systemic, chronic inflammatory disease of the elderly that primarily affects medium- to large-sized arteries including the aorta. Early recognition, evaluation, and treatment are critical because untreated giant cell arteritis can lead to severe bilateral and permanent visual loss. Giant cell arteritis is a clinical diagnosis that is supported by laboratory testing (e.g., erythrocyte sedimentation rate and C-reactive protein) and confirmed by temporal artery biopsy. Corticosteroid therapy should be started immediately before laboratory or pathologic confirmation. Most patients require long-term maintenance steroid treatment lasting months to years.

Clinical Diagnosis of Giant Cell Arteritis

The systemic features of giant cell arteritis are typically headache, neck pain, scalp tenderness, pain in the ear or jaw (especially with chewing food), polymyalgia rheumatica, and constitutional symptoms (e.g., weight loss, fatigue, fever) (Table 1).

Table 1. Typical Features of Giant Cell Arteritis

Reprinted with permission from Lee AG, Brazis PW. Clinical Pathways in Neuro-ophthalmology: An Evidence-Based Approach. New York, NY: Thieme; 1998.

The most common cause of visual loss in patients with giant cell arteritis is anterior ischemic optic neuropathy. A pale and swollen disc (pallid edema) is highly suggestive of giant cell arteritis (Slide 1). Although most patients do not require fluorescein angiography (Slide 2), patients with choroidal filling defects and anterior or posterior ischemic optic neuropathy should be considered for giant cell arteritis until proven otherwise. Less common presentations for giant cell arteritis include nonembolic central retinal artery occlusion, posterior ischemic optic neuropathy, and choroidal or occipital lobe ischemia. The major consideration in anterior ischemic optic neuropathy is differentiating arteritic from nonarteritic disease. The features differentiating arteritic anterior ischemic optic neuropathy from non-arteritic anterior ischemic optic neuropathy are listed in Table 2.

Table 2. Features Suggestive of Arteritic Anterior Ischemic Optic Neuropathy Versus Non-Arteritic Anterior Ischemic Optic Neuropathy

Reprinted with permission from Lee AG, Brazis PW. Clinical Pathways in Neuro-ophthalmology: An Evidence-Based Approach. New York, NY: Thieme; 1998.

Laboratory Testing

Slide 1

Slide 1

Elderly patients suspected of having giant cell arteritis should undergo elevated erythrocyte sedimentation rate testing and a C-reactive protein treatment. Although the erythrocyte sedimentation rate is usually elevated in giant cell arteritis, it can be normal in up to 30% of biopsy-proven giant cell arteritis cases. The normal values for the elevated erythrocyte sedimentation rate are age- and gender-dependent. A formula for determining the maximum elevated erythrocyte sedimentation rate is below.

Typically, I recommend the use of the elevated erythrocyte sedimentation rate and another acute phase reactant, such as C-reactive protein, because the combination of both tests is more sensitive than either test alone. Although other laboratory tests may be abnormal when testing for giant cell arteritis (platelets, other blood factors) they are not as sensitive or specific for giant cell arteritis as the elevated erythrocyte sedimentation rate and C-reactive protein.

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Temporal Artery Biopsy

Slide 2

Slide 2

Tissue confirmation of giant cell arteritis with a temporal artery biopsy is desirable for the following reasons:

  1. The risk of temporal artery biopsy is extremely low and the benefit of a tissue diagnosis outweighs the minimal morbidity for the procedure.
  2. Tissue diagnosis can justify the continued use of steroids for patients who develop complications and prevent the inappropriate or rapid tapering of the steroid.
  3. Confirmation or elimination of the diagnosis in difficult or uncertain cases.

A unilateral or bilateral (sequential or simultaneous) temporal artery biopsy may be performed depending on the pretest likelihood for giant cell arteritis. I perform a unilateral temporal artery biopsy (Slide 3) on the symptomatic side for all patients suspected of giant cell arteritis. Most clinicians consider sensitivity for a unilateral temporal artery biopsy in giant cell arteritis to be approximately 96%. A contralateral temporal artery biopsy is performed if the initial temporal artery biopsy is negative and the post-test likelihood for disease remains high. The biopsy may show active inflammatory infiltrate and giant cells (Slide 3) or, in cases treated prior to the temporal artery biopsy, there may only be evidence of healed arteritis (disruption of the internal elastic lamina).

Treatment

Slide 3

Slide 3

Untreated giant cell arteritis is a medical emergency and visual loss is the most feared complication. Serious systemic complications include cerebral or myocardial infarction or aortic aneurysm, dissection, or stenosis. Corticosteroid therapy should begin immediately before laboratory or histologic confirmation and the goal is to prevent visual loss. Oral prednisone at 1.0 mg/kg to 1.5 mg/kg/d (60 mg to 100 mg per day) is first-line treatment, but intravenous (IV) steroids have been recommended by some authors. Although most patients can be taken off steroids within 12 months, some patients may require prolonged (years) or even lifelong therapy. Chronic corticosteroid therapy is often associated with systemic side effects and consultation with the patient's internist or rheumatologist is recommended. Supplementation with vitamin D and calcium to prevent glucocorticoid-induced osteoporosis is reasonable.The role of aspirin therapy remains unknown but is probably a reasonable consideration.

Although it has not been studied in a head-to-head trial, I consider IV steroids for the following scenarios:

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Acknowledgement: This work was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, NY, and the American Geriatrics Society/John A. Hartford Society Project to Increase Geriatrics Expertise in Ophthalmology.