Giant Cell Arteritis: Causes, Symptoms and Treatment

Giant cell arteritis is an inflammation of the walls of the arteries. Most of the time, it affects the arteries of the head, especially those of the temples. For this reason, giant cell arteritis is sometimes called temporal arteritis.

Giant cell arteritis typically causes headaches, scalp tenderness, jaw pain, and vision problems. Without treatment, this can lead to blindness.

Prompt treatment with corticosteroids usually relieves symptoms of giant cell arteritis and can prevent vision loss. You will probably start to feel better a few days after starting treatment. However, even with treatment, relapses are common.

You will need to see your doctor regularly for check-ups and treatment for side effects from taking corticosteroids.

The most common symptoms of giant cell arteritis are headache and tenderness, usually severe, usually involving both temples. The headache may get progressively worse, come and go, or temporarily disappear.

Typically, the signs and symptoms of giant cell arteritis include:

Severe, continuous headache, usually in the temple area Sensitive scalp Jaw pain when chewing or opening your mouth wide Fever Fatigue Unintentional weight loss Loss of vision or double vision, especially in people who also have pain jaw Sudden and permanent loss of vision in one eye

Pain and stiffness in the neck, shoulders, or hips are common symptoms of a related disorder, polymyalgia rheumatica. About 50% of people with giant cell arteritis also have polymyalgia rheumatica.

If you develop a new, persistent headache or any of the signs and symptoms listed above, see your doctor immediately. If you are diagnosed with giant cell arteritis, starting treatment as soon as possible can usually help prevent vision loss.

Several factors can increase the risk of developing giant cell arteritis, such as the following:

Age.

Giant cell arteritis only affects adults and rarely those under 50. Most people with this disease develop signs and symptoms between the ages of 70 and 80.

Sex.

Women are about twice as likely to develop the disease as men.

Race and geographic region.

Giant cell arteritis is more common in whites of northern European populations or of Scandinavian descent.

Polymyalgia rheumatica.

If you have polymyalgia rheumatica, you are at a higher risk of developing giant cell arteritis.

Family history.

In some cases, the disease can run in the family.

Giant cell arteritis is difficult to diagnose because early symptoms resemble those of other common diseases. For this reason, the doctor will try to rule out other possible causes for the problem.

In addition to asking about your physical symptoms and medical history, your doctor will likely perform a thorough physical exam, paying more attention to the temporal arteries. Many times one or both arteries are tender, have a reduced pulse, and look and feel like a hard cord.

The doctor may also recommend certain tests.

The following tests may be done to diagnose your condition and monitor your progress during treatment.

Measurement of erythrocyte sedimentation rate.

This test, commonly known as erythrocyte sedimentation rate, measures the rate at which red blood cells sink to the bottom of a tube of blood. The rapid fall of red blood cells can indicate inflammation in the body.

C reactive protein.

This test measures a substance that the liver produces when it is inflamed.

They can be used to diagnose giant cell arteritis and to monitor response to treatment. The tests can be the following:

Doppler ultrasound.

This test uses sound waves to produce images of blood flow through blood vessels.

Magnetic resonance angiography.

This test combines the use of

Magnetic resonance imaging

and the use of a contrast medium to produce detailed images of blood vessels. Tell your doctor ahead of time if you are uncomfortable being confined to confined spaces because the test is performed inside a tube-like machine.

Positron emission tomography.

If your doctor suspects that you have giant cell arteritis in large arteries, such as the aorta, they will likely prescribe a

Positron emission tomography

. For this test, a solution containing a tiny amount of radioactive material is used as an intravenous radiotracer. An exploration through

Positron emission tomography

Produces detailed images of large blood vessels and highlights areas of inflammation.

The best way to confirm a diagnosis of giant cell arteritis is to take a small sample (biopsy) from the temporal artery. This artery is near the skin, just in front of the ears and continues to the scalp. The procedure is performed on an outpatient basis under local anesthesia, usually without major discomfort or scarring. The sample is analyzed under a microscope in a laboratory.

If you have giant cell arteritis, the artery often has inflammation that includes abnormally large cells called giant cells, from which the disease gets its name. It is possible to have giant cell arteritis and have a negative biopsy result.

If the results aren’t clear, your doctor may want you to have another temporal artery biopsy on the other side of your head.

The main treatment for giant cell arteritis is to take high doses of a corticosteroid such as prednisone. Since immediate treatment is needed to prevent vision loss, your doctor will likely have you take medication before confirming the diagnosis with a biopsy.

You will probably start to feel better a few days after starting treatment. If you have vision loss before starting corticosteroid treatment, your vision is unlikely to improve. However, the unaffected eye can compensate for some of the vision changes.

You may need to continue taking the drugs for a year or two, or even longer. After the first month, your doctor may begin to gradually decrease the dose until you reach the lowest dose of corticosteroid needed to control inflammation.

Some symptoms, especially headaches, may reappear during this reduction period. This is when many people also develop symptoms of polymyalgia rheumatica. Such flare-ups can usually be treated with small increases in corticosteroid dose. Your doctor might also suggest an immunosuppressive drug called methotrexate (Trexall).

Corticosteroids can cause serious side effects, including osteoporosis, high blood pressure, and muscle weakness. To counter potential side effects, your doctor will likely check your bone density and prescribe calcium and vitamin D supplements or other medications to prevent bone loss.

The United States Food and Drug Administration recently approved tocilizumab (Actemra) to treat giant cell arteritis. It is given as an injection placed under the skin. Side effects include making you more susceptible to infections. More research is needed.