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What is Giant Cell Arteritis?

You might not have heard of Giant Cell Arteritis (GCA) also known as temporal arteritis. The majority of patients first hear about the disease when they are diagnosed. You will have heard of arthritis though, and know that this condition causes painful inflammation (swelling) of the joints. Giant Cell Arteritis (pronounced art-er-it is), is inflammation of the arteries. The itis part of the word actually means inflammation. The underlying cause of GCA is largely unknown, but it is increasingly recognised that there is a genetic link.

Why is inflammation a problem?

People with GCA develop inflammation in the walls of their arteries – caused by an immune reaction – which makes the wall of the artery swell. When we look down a microscope at the swollen arterial wall we can see a lot of white blood cells (cells involved with the immune response) and often we also see “giant cells” which are a particular sort of white cell. Because the wall of the artery is swollen, it can restrict blood flow, which means the areas of the body supplied by that artery do not get enough oxygen. This can cause a wide range of symptoms, such as pain in the head. If this condition goes on for too long without being diagnosed and treated, it can lead to loss of vision, cramp in the jaw muscles when eating, or poor circulation to the arms. Some patients develop inflammation of the large blood vessels exiting the heart that may eventually lead to widening of the aorta known as an aortic aneurysm.

GCA is an autoimmune disorder

Immune reactions are important because they are our body’s way of identifying things that are harmful to us, such as viruses, and sending a message to destroy them. In autoimmune disorders, like GCA, the immune system is mistakenly triggered and white blood cells start to attack healthy tissue. In GCA, this reaction causes inflammation of the arteries, often together with giant cells.

How many people suffer from GCA?

GCA is the most common form of vasculitis in Western countries. It mainly affects people over the age of 50, and is more common in women, in those of Northern European descent, and in those with polymyalgia rheumatica: it is very rare for younger adults to develop GCA. In the UK, about 1 in 4,500 people will be affected. In Leeds, where the TARGET Consortium is based, about five patients per week are referred to our service, of which one-fifth is diagnosed with GCA.

What are the symptoms of GCA?

GCA can present in many different ways. Five well-known symptoms are:

Headache
The most common symptom of GCA is said to be headache, which is reported in around two-thirds of patients. Patients experience the GCA headache in very different ways. Most people report it is not like a “normal” headache that they have experienced previously in their lives, and find it difficult to describe because it’s new for them. It can affect any part of the head, and often does not seem to respond to painkillers, or go away by itself.
Arterial swelling
GCA frequently affects the temporal arteries which are found in the temples (located on either side of the forehead next to the eyes). This swelling of the walls of the artery can sometimes be seen or felt by a doctor. In such cases the artery will usually be tender to the touch.
Scalp tenderness
The headache of GCA is usually accompanied by tenderness of the scalp. Patients can find it very painful to brush their hair, or to put their head on a pillow at night.
Cramping of the jaw when eating
Pain in the muscles of the jaw is quite common in GCA. Sometimes this is what doctors call “jaw claudication” which is when the jaw muscles become painful after eating chewy foods, and then gets better again after rest. This is very similar to angina pain that occurs with exercise. However, sometimes the jaw pain feels more like toothache or just mouth pain. Sometimes the tongue can go into cramp or be painful as well.
Vision problems
GCA can affect the blood supply to the eyes. This can cause a variety of visual symptoms, including temporary or permanent loss of vision. Sometimes only part of the vision is affected. Sometimes double vision can occur. If there is loss of vision in one eye, doctors are keen to treat the patient quickly in order to prevent loss of vision in the other eye. This is a medical emergency and you should seek immediate medical advice from your local eye casualty.

Other symptoms

Often, when GCA patients are asked to think back, they realise they have felt unwell for a while before deciding to visit the doctor. Often GCA starts by feeling “off-colour”, and patients may remember having felt tired, off their food, or achy and stiff. These symptoms of stiffness and achiness can be symptoms of polymyalgia rheumatica (PMR). Quite often PMR symptoms are the first thing patients notice during relapses of GCA. If GCA goes on for a long time before being diagnosed and treated, patients may lose weight or notice fever or sweats. Patients may also feel depressed or frustrated before getting their diagnosis.

We are committed to improving public awareness of GCA and helping patients to understand the signs of GCA

When do I seek medical advice?

GCA requires urgent medical attention and treatment with steroids. If you develop a headache that is not like any headache you’ve had before and it won’t go away – especially if you have noticed jaw pain or changes in your vision – you should see a doctor as quickly as possible. This is particularly important if you’ve noticed loss of vision in one or both eyes, even if the visual loss has got better again. In the UK, this means phoning your GP and asking for an emergency appointment, ringing the NHS helpline 111, or attending the Accident and Emergency department at your local hospital.

We want to make patients aware that GCA is a medical emergency and symptoms should not be ignored

How is GCA diagnosed?

Diagnosing GCA is a challenge because the symptoms are so variable. Headaches are very common in the general population, and the other symptoms of GCA are not well-known. This means that:

In the early stages of disease, patients might ignore their symptoms until they become quite severe.
There are not necessarily enough early indicators for doctors to identify GCA as a possible cause.

It is therefore important that people understand what the disease is and how to spot the signs. A rapid, accurate diagnosis of GCA can be sight saving.

Blood tests

Patients with suspected GCA are often sent for blood tests, such as the C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) or plasma viscosity (PV). These are sometimes called “markers of inflammation” or “inflammatory markers”. When these markers are high, it can indicate that the body is reacting to something. However, high inflammatory markers are seen in a wide range of medical conditions so are not specific to GCA.

Eye tests

Patients with visual symptoms that are thought to be due to GCA should see an eye specialist (ophthalmologist) urgently. These tests will be different those you have when you see your high-street optician. The ophthalmologist will see if there are any signs of loss of blood supply to the eye, or whether there might be alternative explanations for loss of vision. This usually involves having special drops placed in your eyes to make your pupils temporarily larger so that the ophthalmologist can look into the back of the eye using a special piece of equipment called a slit-lamp. More detailed examinations of the nerves or blood vessels supplying the eye are sometimes needed.

Temporal artery biopsy

A temporal artery biopsy can confirm the diagnosis of GCA. This test is carried out by a surgeon under a local anaesthetic. A small cut is made into the skin overlying the temporal artery, and a small part of the temporal artery is taken out and sent to the laboratory to be looked at under a microscope. After this, you will usually have some stitches which, after about a week, are either removed at your local doctor’s surgery, or will dissolve on their own. This procedure usually takes around twenty minutes. It should not be painful. You would usually be left with a small scar on the side of your head where the cut has been made.

Vascular ultrasound

The swelling of the artery wall in GCA may be seen using ultrasound. Sometimes this test is done by the doctor evaluating your case, and sometimes it is done by a vascular technician (sonographer). This test is similar to the scans used to look at a baby in the mother’s womb. The person doing the scan holds a plastic stick which makes sound waves that are too high to hear. They place water-based gel on your skin and then place the plastic stick on top of that. The stick listens for reflections of the sound waves. The most common areas scanned for the diagnosis of GCA are the temporal arteries (on the side of the head) and the axillary arteries (under the armpits) because the arteries in those places are close to the surface of the body and the sound waves can reach them. This test does not involve any needles or cuts. This test is new and not available in all hospitals at the present time.

Other vascular imaging

Sometimes, in order to study arteries that are deeper inside the body (such as the aorta) other vascular imaging tests are carried out. Which test requested depends a lot on the expertise available in the hospital concerned, your symptoms, and whether you have already started taking steroids. Magnetic resonance imaging, CT angiography and PET/CT scans can all be useful in selected patients. These tests are not usually helpful for detecting inflammation in the blood vessels of the head. For most of these tests you will be asked to lie still for at least half an hour, and sometimes you will receive an injection into a vein to help the blood vessels look clearer on the scans.

We are trying to find one simple blood test that can diagnose GCA quickly and with 100% certainty

How is GCA treated?

Once a diagnosis is made, high-dose steroids are given. Sometimes this treatment is started while the doctors are waiting for all the test results to come back.

Immediate treatment

The main treatment for GCA is steroid medication (are also called corticosteroids or glucocorticoid). They are usually given in tablet form (in the UK they are called prednisolone and in the US prednisone) although in the early stages sometimes steroids are given into the vein as an infusion to get a high dose into the body very quickly. At the start of treatment, a high dose of steroids are needed.

Prednisolone dose tapering

Over time (weeks to months) the dose of steroids is gradually reduced. We call this “dose tapering”. It has to be gradual because the body normally makes a small amount of its own steroids to help maintain normal functioning, but with long-term steroid treatment the body adapts and stops making steroids of its own. If steroid medication is stopped too suddenly, the body might be short of steroids and this can make you very ill. For this reason, people taking long-term steroids shouldn’t stop taking their steroids suddenly, and should work with their medical team when planning dose adjustments.

Are steroids a cure or do they just keep the disease under control?

As far as we know, the main role of steroids is to dampen down and then keep inflammation under control until the immune system stops reacting to the blood vessels. Perhaps half of all patients with GCA appear able to stop steroid treatment completely after a year or two without any further return of the disease.

Follow-up

It is important to remain under medical follow-up for as long as you are taking steroids for GCA. You would normally be sent regular appointments, and you should keep in touch with your GP. It is important to make the most of your appointments and ask any questions you may have about your disease, its treatment and what you can do to help yourself, including diet and exercise. If you experience a return of GCA symptoms during any part of your treatment, contact your doctor immediately for re-assessment. Please try to have an agreed plan in place with your doctor so you know what to do if your symptoms return.

We work with doctors to improve the speed of GCA diagnosis, and to provide gold-standard guidance for GCA treatment pathways

What are the complications of GCA and side effects of GCA treatment?

Complications associated with GCA

In GCA, the blood vessel walls become thickened by the inflammatory process and this can block the flow of blood. This is what can cause visual loss and jaw cramps. Over time and with treatment, the thickening of the arteries reduces. However, the inflammatory process may also cause damage to the vessel wall and in the long-term can cause dilatation (widening) or aneurysm of the larger arteries.

Vision loss and blindness

Blindness is one of the most feared complications of GCA. It is said that around one in five people with GCA will experience some degree of visual loss. This can range from some vision loss in one eye to total blindness.

Aortic complications

The aorta is the major artery that carries blood from the heart to the rest of the body. In GCA, inflammation often affects the wall of the aorta as well as the temporal arteries. Inflammation can also affect the branches that come off from the aorta, especially the branches that supply blood to the arms. It is reported that patients with GCA are at a slightly higher risk of developing an aortic aneurysm (widening of the aorta). It usually affects the part of the aorta inside the chest, meaning it would not be picked up by ultrasound tests designed to look at the part of the aorta inside the abdomen (stomach).

Complications associated with GCA treatment

Most patients with GCA need steroid (prednisolone) treatment for at least a year and often much longer. Because of this, often there is a need to take further medication to reduce the risk of any side-effects of steroid treatment. For example, patients who take long-term steroids are more likely to break a bone if they fall over. Some medications can help reduce this risk.

Prednisolone

Side-effects from prednisolone relate to the dose that is taken. The higher the dose, the higher the risk of side-effects. Your doctor will consider whether additional treatments are needed to prevent some of these side effects. During tapering of the dose, most of these side-effects should get better. The most noticeable side-effects at an early stage are usually related to the fact that prednisolone mimics cortisol which is a hormone that your body produces as part of the stress response. Difficulty sleeping and feeling “jittery” is quite common. Mood change may occur (either high or low) with high-dose steroids. It is important to talk about any side-effects with your doctor.

Other side effects of prednisolone include:

Increased appetite, leading to weight gain
Increased blood pressure (hypertension)
Mood changes, such as becoming aggressive or irritable
Increased risk of breaking bones (osteoporosis)
Muscle weakness (myopathy)
Skin thinning
Bruising easily
Problems with wound healing
Stomach ulcers
Increased risk of infection
Diabetes
Glaucoma and Cataracts

If you take steroids for more than three weeks, it is useful to carry a steroid card. These are often provided by pharmacists in the UK. It’s important to carry this with you at all times as it will explain that you’re regularly taking steroids and that your dose shouldn’t suddenly be stopped: this is very important in the event of a sudden illness, accident or emergency. For more information about the side effects of prednisolone please follow this link.

We are researching the side-effects of the drugs used to treat GCA, and are working with other experts and pharmaceutical companies to explore the use of new drugs that may be equally effective but carry fewer side-effects

For more information about GCA, and about support that may be available please follow this link

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