Giant Cell Arteritis, the Headache and Jaw Pain That Can Threaten Sight

Some causes of sight loss begin in the eye. This one begins in the blood vessels of the head. Giant cell arteritis is a condition that inflames the arteries in the head and neck, and when the blood supply to an eye is reduced, sight can be lost in that eye. The first signs are often a new headache, a tender scalp or a sore jaw, and they can look like other, less serious problems. This guide explains the warning signs of giant cell arteritis, why it is treated as an emergency and what treatment involves. It draws on the information published by RNIB.

What giant cell arteritis is

Giant cell arteritis, often shortened to GCA, affects large and medium sized arteries in the body. It particularly affects the arteries in the neck and head. It is also called temporal arteritis, because it often involves the temporal artery, which runs at the side of the forehead, at the temple.

In GCA the inner walls of the arteries become swollen or thickened. This is caused by inflammation, which can include inflammatory cells called giant cells. The swelling restricts the blood flowing through the artery, so the parts of the body that the artery feeds may not receive the blood they need. RNIB explains that if the blood flow to the eye is reduced, sight can be lost in that eye. For this reason giant cell arteritis is considered an emergency.

Who gets it

The cause is not known. It is thought to be an autoimmune condition, which means the body’s own defences turn against it. GCA rarely affects people under the age of 50. It usually starts from about 60, and it is most common in people over 70. Women are affected more often than men.

The rest of this guide describes what to look for.

The early warning signs

RNIB describes three signs that often come first. Most people with GCA have a headache before anything else.

• A new headache. One of the first signs is a new onset of frequent or constant headache. It can be severe, and it can arrive suddenly or build up gradually. It is often at the side of the forehead, at the temples, and it may be on one or both sides of the head.
• A tender scalp or temple. The temples and scalp may feel sore to the touch. Combing or brushing the hair can become uncomfortable. The temporal artery lies close to the skin just in front of the ear and continues up into the scalp, and it can become thickened and more prominent.
• Jaw pain when chewing. This is a cramp-like pain in the jaw that can make a person stop eating, and it eases when the chewing stops. The medical name is jaw claudication. It happens because the muscles used for chewing are short of blood. The same shortage can cause pain in the tongue, mouth or ear.

RNIB’s advice is clear. A person with any of these symptoms should contact a doctor urgently. This is not something to wait out.

Other symptoms that can go with it

Some people also have more general symptoms. These include weight loss, tiredness, flu-like symptoms, night sweats, fever and low mood. The signs of GCA can look like the signs of other conditions, and that makes it difficult to diagnose. It is another reason to describe every symptom to the doctor, including the ones that seem unrelated to the eyes.

How it can affect the eyes

RNIB says that around 30 in 100 people with GCA have some disturbance of vision. For most of them the headache comes first. For some, a problem with vision is the first sign. These are the eye symptoms it describes.

• Temporary loss of vision in one eye. All sight in one eye may disappear for a time and then return. The medical name is amaurosis fugax.
• Double vision. The muscles that move the eyes can be affected. The double vision may last seconds or minutes, or it may stay.
• Permanent loss of vision. GCA can cause severe permanent loss of sight in one eye. Without immediate treatment it can also lead to sight loss in the other eye, and this often happens within a week of the first eye developing symptoms.

The most common cause of the sight loss is damage to the optic nerve. It may also be due to a loss of blood supply to the retina. In rare cases a person with GCA can have a stroke that causes sight loss.

The encouraging part is the last line of RNIB’s explanation. Prompt treatment can stop the other eye from becoming involved.

Sudden loss of vision is an emergency

RNIB states this plainly. Sudden loss of vision is an emergency and should always be investigated immediately by an ophthalmologist, an eye specialist doctor. Three points follow from it.

• The sight should be assessed even if it comes back after a while.
• It should be assessed even if there are none of the other symptoms of GCA. Some people lose their sight without noticing any other symptom first.
• The aim is to be seen quickly enough that, if treatment is needed, it can be given as soon as possible.

RNIB lists the routes to an urgent review in the United Kingdom, such as an eye emergency department, a family doctor or a hospital emergency department. Wherever you live, the principle is the same. Go to the place that can examine eyes fastest, and do not wait for an appointment. A guide to what a dilated eye exam checks shows how the back of the eye is examined once you are there.

How doctors diagnose it

If GCA is suspected, treatment is started straight away, even before all the test results are back. The aim is to reduce inflammation as quickly as possible and to stop the flow of blood in other arteries from being restricted. Several checks are then made.

• An eye examination. Vision is measured on a chart, and the specialist looks for signs of GCA. There may be a scan of the optic nerve and retina called optical coherence tomography.
• An examination of the artery at the temple, checking the pulse and whether the artery has thickened.
• Blood tests that look for signs of inflammation. These tests do not show exactly where the inflammation is, and in some people the results are inconclusive. Other tests, such as a temporal artery biopsy or an ultrasound scan, are used to confirm the diagnosis.

What the treatment involves

When GCA is suspected, emergency treatment with high-dose steroids is started immediately. Steroids reduce inflammation in the body. They may be given as tablets, or as a drip into a vein that is usually given for three days. The high doses are meant to bring down the swelling in the arteries. This helps to prevent sight loss. If sight has already been lost, the aim is to protect the other eye. Treatment also helps to prevent rare complications, such as stroke.

The course is long. RNIB says people will probably take steroid tablets for between 18 months and two years, or even longer, with the dose reduced gradually as the inflammation settles. A small number of people need steroids for the rest of their lives.

Other medicines may be added. RNIB names two. Tocilizumab is a biological medicine that blocks a protein linked to inflammation, and it can reduce the amount of steroid needed. It may be used when GCA flares up as steroids are reduced. Methotrexate is a medicine that suppresses the immune system and reduces inflammation. It may be prescribed after a relapse, or when steroid side effects are a problem. Decisions about these medicines belong to the specialist.

A link with muscle pain and stiffness

About half of the people with GCA also have a condition called polymyalgia rheumatica, or PMR. It causes inflammation in the blood vessels that feed the larger muscles, mainly in the shoulders, neck, hips and back. These muscles can be painful and stiff, especially in the morning. A person can have GCA without PMR. Both conditions are treated with steroids.

Managing with changed sight

For some people both eyes are affected. For others the eye that lost sight was the good eye, and the other eye already had a problem. RNIB says there is a lot that can be done to make the most of remaining vision. Things can be made bigger, lighting can be brighter and colour can be used to make objects easier to see. A low vision assessment lets a person discuss practical problems with a specialist, who can look at magnifiers, lighting, colour contrast and other adaptations. Assistive technology can also help with work, hobbies and daily tasks.

It is normal to worry about the future after a diagnosis. Talking to someone outside the family can help, and a family doctor can advise.

If you or a relative notices these signs

• Write down what has happened and when it started, including the headache, scalp pain, jaw pain and any change in sight.
• Do not wait for the next routine appointment, and do not wait to see whether the symptoms pass.
• If sight is lost, even briefly, get an eye examination urgently.
• Take a list of every medicine you use.

The wider picture on sight loss that can be avoided is explained in a guide on why most blindness is avoidable. More guides on eye conditions are collected in the eye care and treatment section.

Asking for help

If someone you know has these signs, get them seen today. For general questions about giant cell arteritis, ask PEBS about giant cell arteritis. Readers who wish to give can review ways to support patient care.

Usually the warning signs arrive before the sight is affected, and that gap is the chance to protect the eyes.

This article is general information and does not replace an examination by an eye doctor.