Diseases & Conditions

Giant cell arteritis

Giant cell arteritis (GCA) is an inflammation of the lining of your arteries — the blood vessels that carry oxygen-rich blood from your heart to the rest of your body. Most often, it affects the arteries in your head, especially those in your temples. For this reason, giant cell arteritis is sometimes called temporal arteritis or cranial arteritis.

Giant cell arteritis frequently causes headaches, jaw pain, and blurred or double vision. Blindness and, less often, stroke are the most serious complications of giant cell arteritis.

Prompt treatment with corticosteroid medications usually relieves symptoms of giant cell arteritis and may prevent loss of vision. You should start feeling better within days of starting your treatment.

The most common symptoms of giant cell arteritis are head pain and tenderness — often severe — that usually occurs in both temples. Some people, however, have pain in only one temple or the front of the head.

Signs and symptoms of giant cell arteritis can vary. For some people, the onset of the condition feels like the flu — with muscle aches (myalgia), fever and fatigue, as well as headache.

Generally, signs and symptoms of giant cell arteritis include:

• Persistent head pain and tenderness, usually in your temple area
• Decreased visual acuity or double vision
• Scalp tenderness — it may hurt to comb your hair or even to lay your head on a pillow, especially where the arteries are inflamed
• Jaw pain (jaw claudication) when you chew
• Pain and stiffness in your neck, arms or hips — usually worse in the morning before you get out of bed
• Sudden, permanent loss of vision in one eye
• Fever
• Unintended weight loss

The pain and stiffness in the neck, arms or hips are common symptoms of a related disorder, polymyalgia rheumatica. Approximately half the people with GCA also have polymyalgia rheumatica.

When to see a doctor
If you develop a new, persistent headache or any of the problems listed above, see your doctor without delay. If you're diagnosed with GCA, starting treatment as soon as possible can usually help prevent blindness.

Your arteries are pliable tubes with thick, elastic walls. Oxygenated blood leaves your heart through your body's main artery, the aorta. The aorta then subdivides into smaller arteries that deliver blood to all parts of your body, including your brain and internal organs.

With giant cell arteritis, some of these arteries become inflamed, causing them to swell. Just what causes these arteries to become inflamed isn't known.

Although almost any large or medium-sized artery can be affected, swelling most often occurs in the temporal arteries, which are located just in front of your ears and continue up into your scalp. In some cases, the swelling affects just part of an artery, with sections of normal vessel in between.

Treatment for GCA consists of high doses of a corticosteroid drug such as prednisone. Because immediate treatment is necessary to prevent vision loss, your doctor is likely to start medication even before confirming the diagnosis with a biopsy.

You should start feeling better within just a few days, but you may need to continue taking medication for one to two years or longer. After the first month, your doctor may gradually begin to lower the dosage until you reach the lowest dose of corticosteroids needed to control inflammation as measured by sed rate and CRP tests. Some of your symptoms may return during this tapering period.

What are corticosteroids?
Corticosteroids are powerful anti-inflammatory drugs whose effects mimic those of hormones produced by your adrenal glands. The drugs can effectively relieve pain, but prolonged use — especially at high doses — can lead to a number of serious side effects.

Older adults, who are most likely to be treated for giant cell arteritis, are particularly at risk because they're more prone to certain conditions that also may be caused by corticosteroids. These include:

• Osteoporosis
• High blood pressure
• Muscle weakness
• Cataracts

Other possible side effects of cortisone therapy include:

• Weight gain
• Increased blood sugar levels, sometimes leading to diabetes
• Thinning skin and increased bruising
• Decreased function of your immune system

To counter the potential side effects of corticosteroid treatment, your doctor is likely to monitor your bone density and may prescribe calcium and vitamin D supplements or other medications to help prevent bone loss. He or she is also likely to monitor your blood pressure and may recommend an exercise program, diet changes and sometimes medication to keep blood pressure within a normal range. Most side effects go away when the corticosteroid treatment is stopped.

New treatments
Researchers are trying to find therapies that work as well as corticosteroids but cause fewer side effects.

One drug under investigation is methotrexate, which doctors often use to treat certain cancers and some inflammatory conditions such as rheumatoid arthritis. The hope is that by using both methotrexate and prednisone to treat people with GCA, it would be possible to use less prednisone. But preliminary research results are conflicting, so more research is needed.

A small study suggested that azathioprine (Imuran) may be helpful in the treatment of giant cell arteritis when used along with smaller doses of prednisone. Additionally, some case reports suggest that tumor necrosis factor (TNF) blockers, such as etanercept (Enbrel), may be helpful. However, larger studies need to be done to confirm the effects of all of these medications.

Ask your doctor about taking low-dose aspirin (anti-platelet therapy). Taken on a daily basis, it may reduce the risk of blindness and stroke.