Temporal Arteritis (Giant Cell Arteritis)

 

What Is It?

Giant cell arteritis, also called temporal arteritis, is a disease in which the blood vessel branches supplying the eye, scalp and face become inflamed and narrowed. Early diagnosis and treatment is essential because vision loss is a complication of this disease. Larger blood vessels, including the aorta and its branches, also may be involved, leading to aneurysm and even rupture years later.

This disorder develops almost exclusively in people older than age 55 and most commonly affects people in their 70s and 80s. It is relatively rare, occurring in an estimated two people in 1,000.

The cause of giant cell arteritis is unknown, although there has been speculation that in some people, the body has developed antibodies to the lining of blood vessels, perhaps in response to an infectious agent.

Symptoms

A dull, throbbing headache, often at one or both sides of the forehead, is typical, although it may be localized anywhere over the head or into the neck. Other common symptoms include one or more of the following:

  • Fever
  • Jaw pain after chewing (jaw claudication)
  • Loss of appetite
  • Fatigue
  • Dry cough
  • Tenderness of the scalp or temples
  • Blurring or loss of vision in one eye
  • Aching in the shoulders, upper arms or hips and/or upper thighs, especially in the morning (typical of polymyalgia rheumatica, an arthritic condition associated with giant cell arteritis)

Diagnosis

Your doctor may suspect this disorder if you are an older adult with a new headache. He or she will begin by asking you for a detailed description of your symptoms, past medical problems and medications. Next the doctor will conduct a complete physical examination with a particular focus on the temporal arteries, located just in front of the ear, above the jaw, and other blood vessels. Other causes of headache or other symptoms will be considered, and relevant tests will be recommended. However, if giant cell arteritis is still a strong concern after these tests, a blood test called the erythrocyte sedimentation rate (ESR), will be ordered because it is almost always elevated in this condition. A biopsy of the temporal artery generally is required to confirm the diagnosis. In this procedure, a small piece of the blood vessel will be removed under local anesthesia and will be examined in a laboratory.

Expected Duration

Giant cell arteritis typically lasts several years. The course of untreated disease has not been studied, but after effective therapy is started, improvement is usually prompt. Some people are able to discontinue treatment after six months, but longer treatment over two to three years is more usual. Relapses after prolonged periods of remission occasionally occur.

Prevention

There is no known way to prevent this disease, although vision loss can be prevented by prompt diagnosis and treatment.

Treatment

Giant cell arteritis is a serious disease requiring prompt medical attention. Treatment is usually with high doses of prednisone, a corticosteroid, and most people feel better within a few days. Medication is continued at this high dose for approximately one month and then tapered gradually over the following months to the lowest possible dose or is discontinued. It is not rare to require at least six months of therapy, and often treatment is continued for a year or more because many people experience symptoms typical of polymyalgia rheumatica (aching in the shoulders, hips or both) or recurrent headaches as the prednisone dose is reduced. Some studies have found that another medication, methotrexate (Folex, Rheumatrex), may reduce the dosage of corticosteroids required and the length of time they need to be taken, although more recent studies have not confirmed this effect. As a result, some physicians prescribe methotrexate as a “steroid-sparing” agent in the hopes of reducing the overall exposure to corticosteroids.

When To Call A Professional

If you have any symptoms suggestive of giant cell arteritis, especially a new headache, unexplained fever or visual symptoms, you should contact your doctor.

Prognosis

If there has not yet been vision loss, the prognosis is excellent. However, once vision loss develops, it is usually permanent. If the aorta or nearby branches are involved, the prognosis may be worse, because these blood vessels may enlarge or even rupture. However, most complications related to giant cell arteritis develop as a complication of steroid therapy (including osteoporosis, diabetes and weight gain), rather than as a result of the disease itself.

Johns Hopkins patient information

Last revised:

Diseases and Conditions Center

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All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.