Giant Cell Arteritis

Signs and symptoms of GCA may begin quite abruptly or sometimes gradually over a number of months before becoming clinically recognizable. Its classic symptoms relate to the inflammation of, and reduced blood flow through, the involved arteries. Most patients present with bilateral headache, often complaining of scalp pain with normally non-noxious stimuli, such as brushing their hair. Transient visual loss, or even permanent visual loss, may result from involvement of the posterior ciliary, ophthalmic, and retinal arteries. Painful cramping or claudication often occurs with the use of the jaw while chewing or on movement of the tongue. Many patients have associated systemic symptoms, such as fever, malaise, sweating, and weight loss. Examination may reveal that the temporal and occipital vessels are firm, tender, and pulseless. Sausage-shaped thickenings or nodularity may be palpable along the vessel wall.

The incidence of GCA increases with advancing age and must be considered in all patients older than 50 years who develop a new headache, have a change in their previous headache characteristics, or have acute-onset transient visual loss. Polymyalgia rheumatica (PMR) is an overlapping disease, and symptoms of PMR are found in more than one third of biopsy-proven cases of GCA. These PMR symptoms include neck pain, morning stiffness, and myalgias in shoulder and pelvic muscles.

Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are nonspecific markers of inflammation that, when elevated—often to very high levels (60 to 120 mm/hr for ESR and greater than 40 for CRP)—are supportive of the diagnosis of GCA. Sedimentation rate is reported to be normal in about 5% of patients with GCA. Normochromic anemia, low albumin, and thrombocytosis are often present.

Diagnosis can only be established with certainty by biopsy of the temporal artery and demonstration of focal inflammation, giant cells, and interruption of the internal elastic lamina. Of importance, none of the testing options have a sensitivity of 100%. Because vessel involvement can be segmental, the biopsy may also be falsely negative. If the biopsy is negative but the clinical suspicion remains high, such as in an elderly patient with a new headache and jaw claudication or systemic symptoms, other tests should be performed, looking for signs of vessel inflammation. These would include another site for artery biopsy, such as the contralateral temporal artery or posterior occipital scalp artery, a magnetic resonance angiogram, duplex ultrasonography, or a positron emission tomography (PET) scan.

When unrecognized and untreated, GCA can lead to a variety of complications. The most devastating is sudden permanent unilateral or sequential bilateral vision loss from anterior ischemic optic neuropathy (AION) or retinal artery occlusion, cerebrovascular ischemia—more commonly in the vertebral basilar system, or even a myocardial infarction. Involvement of the aorta very rarely may lead to aortic dissection or aneurysm. Prompt treatment with corticosteroids is required to prevent permanent sequelae, especially visual loss. Headache and systemic symptoms usually improve within 48 hours of starting treatment, but visual loss and ischemic complications are often irreversible. Biopsy should be obtained within 24 hours of starting steroids.

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