Giant Cell Arteritis (Temporal Arteritis)

What is giant cell arteritis and who does it affect?

Giant cell arteritis (GCA) is a condition which causes inflammation on the inside of some arteries (blood vessels). It is called 'giant cell' because abnormal large cells develop in the wall of the inflamed arteries. The arteries commonly affected are those around the head and neck area. One of the arteries that is commonly affected is the temporal artery. (You have a temporal artery on each side of the head. They are under the skin to the sides of the forehead - the temple area.) Therefore, the condition is sometimes called 'temporal arteritis'. Several arteries may be affected at the same time.

GCA is uncommon and mainly affects people over the age of 60. It rarely affects people under 50. Women are more commonly affected than men. The cause is not known.

What are the symptoms of giant cell arteritis?

Symptoms can vary, and may depend on which artery or arteries are mainly affected.

• Headache is the common symptom. It occurs in about two-thirds of people with GCA. This typically develops suddenly over a day or so, but it sometimes develops gradually over several days or weeks. The headache can be one sided, or on both sides. Typically, it is mainly towards the front and sides of the head.
• Tenderness of the scalp over the temporal arteries is common. You may be able to feel one or both of the inflamed temporal arteries under the skin, or see them in a mirror.
• Other symptoms may occur if the arteries going to these parts of the body are inflamed, become narrowed, and reduce the blood supply to these areas. These include:
  • Pain in the jaw muscles (jaw claudication) while eating or talking. This occurs in nearly half of affected people. The pain eases when you rest the jaw muscles.
  • Visual disturbances: permanent partial or complete loss of vision in one or both eyes occurs in up to 1 in 5 affected people, and is often an early symptom. People who are affected typically report a feeling of a shade covering one eye, which can progress to total blindness. The eye is not painful. If untreated, the second eye is likely to become affected within 1-2 weeks, although it can be affected within 24 hours. Urgent treatment is therefore essential. A temporary loss of vision in one eye, or diplopia (double vision) may occur as a 'warning' symptom before any permanent visual loss.
• Some general symptoms also commonly occur. These include: tiredness, depression, night sweats, fever, loss of appetite, and weight loss. These may develop gradually and may be present for weeks or even months before a specific symptom such as headache or visual loss develop.

Polymyalgia rheumatica - is often also present

Up to half of people with giant cell arteritis develop a related condition called polymyalgia rheumatica (PMR). If this develops it often occurs at the same time, but may occur before or after the development of GCA. The typical symptoms of polymyalgia rheumatica are pain, tenderness and stiffness of muscles around the shoulders and upper arms, and sometimes around the hips and neck. Polymyalgia rheumatica is due to inflammation in the affected muscles, but the cause is unknown. The treatment is similar for both conditions. Treatment for polymyalgia rheumatica is usually very effective. (See a separate leaflet called Polymyalgia Rheumatica for details.)

What are the possible complications of giant cell arteritis?

Complications are much less likely to occur if treatment is started soon after symptoms begin. Possible complications include the following:

Blindness in one or both eyes

If an affected artery becomes very inflamed, the blood supply going down that artery can become blocked. The most common arteries this affects are the small arteries going to the eye. If one of these arteries becomes blocked it can cause permanent, serious visual problems, even blindness, in the affected eye. Total or partial loss of vision may occur in up to 1 in 5 people with untreated giant cell arteritis. Once vision is lost, there is little chance of recovery of vision, even with treatment. Therefore, treatment is aimed at preventing visual loss, or if visual loss has occurred in one eye, to prevent loss in the other eye. However, even with treatment, visual loss occurs in up to 1 in 20 cases.

Problems related to other arteries being affected

Other serious complications sometimes develop if the inflammation occurs in other arteries. For example, a heart attack, an aortic aneurysm, a stroke, damage to nerves, or deafness (caused by a blocked artery in the brain).

Do I need any tests?

A blood test can detect if there is inflammation in your body. (This is the erythrocyte sedimentation rate (ESR) test or the C-reactive protein (CRP) test.) If the blood test shows a high level of inflammation, and you have the typical symptoms, then GCA is likely. However, the blood test is not specific for GCA (it can also be high in other 'inflammatory disorders'.) Also, some people with GCA have a normal blood test.

To confirm the diagnosis a doctor may take a small part of the temporal artery (a biopsy) to look at under a microscope. If you have GCA a doctor can see the inflammation and abnormal giant cells in the sample of the artery wall.

What is the treatment for giant cell arteritis?

If GCA is suspected, treatment is usually started straight away - even before a biopsy can confirm the diagnosis. The main aim is to reduce the risk of possible complications. The second aim is to relieve the headache and any other symptoms.

Steroid tablets

A steroid medicine such as prednisolone is the usual main treatment. Steroids work by reducing inflammation. After starting treatment, symptoms usually ease within a few days.

A high dose of steroid is started at first, usually about 60 mg per day. This is then reduced gradually to a lower 'maintenance' dose. It may take several months to gradually reduce the dose. The maintenance dose needed to keep symptoms away and prevents complications varies from person to person. Usually it is around 10 mg per day.

In some people the condition goes away after 2-3 years, so the steroid tablets may be able to be stopped after 2-3 years. This should always be done under supervision of a doctor. However, many people need treatment for several years, sometimes for life.

Low dose aspirin

In addition to a steroid tablet, a low daily dose of aspirin is usually advised. 75 mg daily is the usual dose. The low dose of aspirin helps to prevent heart attacks and strokes. (As mentioned above, there is an increased risk of developing a heart attack or stroke if you have GCA. However, many people over the age of 50 are already taking low dose aspirin with the aim of reducing the risk of a heart attack and stroke.)

A proton pump inhibitor

Taking both a steroid and aspirin can greatly increase your risk of developing a stomach ulcer. If you take this combination of drugs it is commonly advised that you also take a drug to reduce the acid in your stomach. The aim is to prevent the serious complication of a bleeding stomach ulcer from developing. Proton pump inhibitors (PPIs) are a class (group) of drugs that work on the cells that line the stomach, reducing the production of acid. They include: esomeprazole, lansoprazole, omeprazole, pantoprazole and rabeprazole, and come in various different brand names. One of these will normally be advised if you take a steroid and aspirin.

A drug to prevent osteoporosis

This may be advised - see below for the reasons.

Some other points about steroid tablets

• Do not stop taking steroid tablets suddenly. It probably does no harm if you forget to take the odd tablet. However, once your body is used to steroids, if you stop the tablets suddenly you may get serious withdrawal effects within a few days.
• Do not take anti-inflammatory painkillers whilst you take steroids unless advised by a doctor. The two together increase your risk of developing a stomach ulcer. As mentioned above, you will normally be advised to take low dose aspirin, but avoid other anti-inflammatory painkillers.
• Most people who take regular steroids carry a 'steroid card'. This gives details of your dose, condition, etc, in case of emergencies.
• If you are ill with other conditions, or have surgery, the dose of steroid may need to be increased for a short time. This is because you need more steroid during physical stress.

Side-effects

The risk of developing side-effects from steroids is increased with higher doses. This is why the dose used is the lowest that keeps symptoms away. Possible side-effects from steroids include the following:

• Osteoporosis ('thinning of the bones') - but you can take a drug to help protect against this if you are at increased risk. For example, if you are 65 or older, or have a history of fractures, you should take a drug to help protect against osteoporosis. Your doctor will advise. If you are less than 65 and do not have a history of fractures you may be offered a special scan which measures bone density (a DEXA scan). If your bone density is below a certain level you may be offered a drug to protect against osteoporosis.
• Increased chance of infections - in particular, a severe form of chickenpox and measles. Note: most people have had chickenpox in the past and are immune to it. Also, most people have either had measles or have been immunised against it and are immune. But, if you have not had chickenpox or measles (or measles immunisation), keep away from people with measles, chickenpox, or shingles (which is caused by the same virus as chickenpox). Tell a doctor if you come in contact with anyone with these conditions if you are unsure about your medical past history.
• Weight gain.
• Increase in blood pressure. So have your blood pressure checked regularly. It can be treated if it becomes high.
• High blood sugar which may mean extra treatment if you have diabetes. Steroids may occasionally cause diabetes to develop. If you take long-term steroids, your doctor may arrange a yearly blood sugar test to check for diabetes. In particular, if you have a family history of diabetes.
• Skin problems such as poor healing after injuries, thinning skin, and easy bruising. Stretch marks sometimes develop.
• Muscle weakness.
• Mood and behaviour changes. Some people actually feel better in themselves when they take steroids. However, steroids may aggravate depression and other mental health problems, and may occassionally cause mental health problems. If this side effect occurs it tends to happen within a few weeks of starting treatment and is more likely with higher doses. Some people even become confused, irritable and may even develop delusions and suicidal thoughts. These mental health effects can also occur when steroid treatment is being withdrawn. Seek medical advice if worrying mood or behaviour changes occur.
• An increased risk of developing cataracts.
• An increased risk of duodenal and stomach ulcers. Tell your doctor if you develop indigestion or abdominal (stomach) pains.

Although the above points have to be mentioned, do not be put off about steroids. The relief of symptoms, and the prevention of serious complications, usually outweighs the risk of side-effects from the doses of steroids used for this condition.

Further help and information

PMR and GCA Support

10 Castleroy Road, Broughty Ferry, Dundee, Scotland DD5 2LQ
Tel: 01382 778 596 Web: www.pmr-gca.org.uk
A support group based in Tayside, Scotland, for people with polymyalgia rheumatica and/or giant cell arteritis (also known as temporal arteritis). Although the group is locally based in Tayside, their website has a lot of information about these conditions which a wider audience would benefit from.

PMR-GCA UK North East Region Support Group

Tel: 01287 623334
Web: www.pmr-gca-northeast.org.uk
Offers support and information for people with polymyalgia rheumatica and/or giant cell arteritis.