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Giant cell arteritis – the condition which can cause sudden blindness with clear symptoms a doctor should be looking for

Most people suffer from headaches, neck pains, fever and tiredness at times and put it down to simply being ‘under the weather’.

However, on occasions such symptoms can be an indication that something more serious is wrong – especially when they persist.

One such condition our clinical negligence team has come across with symptoms many patients don’t immediately realise to be serious is giant cell arteritis (GCA).

A condition which causes swelling of the temporal arteries, it should be considered by GPs – especially people aged over 60 – and treated urgently.

Preventable with early diagnosis and prompt treatment with steroids, delays in recognising the condition can lead to irreversible loss of vision.

Sadly, this is what happened to our client Jane Higgins, who despite going to her doctor three times over a three-week period with symptoms of continuing headaches, a stiff neck and pain in her jaw, was not referred to hospital for specialist review.

Why is it called giant cell arteritis (GCA)?

It is called ‘giant cell’ arteritis 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, which are found on each side of the head 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.

What are the common symptoms of GCA?

  • Headache is the common symptom and 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 and typically is mainly towards the front and sides of the head.
  • Tenderness of the scalp over the temporal arteries is also common and you may be able to feel one or both of the inflamed temporal arteries under the skin, or see them in a mirror. They may look swollen or bruised and may have a rash over them.
  • Pain in the jaw muscles (jaw claudication) while eating or talking. This occurs in nearly half of affected people.
  • Permanent partial or complete loss of vision in one or both eyes occurs in up to one in five 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. If untreated, the second eye is likely to become affected within one to two weeks, although it can be affected within 24 hours. Urgent treatment is therefore essential. A temporary loss of vision in one eye or double vision may occur as a ‘warning’ symptom before any permanent vision loss.
  • Other general symptoms include tiredness, night sweats, fever, loss of appetite, and weight loss.

 Why can GCA lead to blindness?

The most common arteries giant cell arteritis 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 20 per cent of people with untreated GCA. 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.

It is because of these risks that GCA  is a condition that warrants urgent treatment and diagnosis.

Once GCA is suspected, immediate treatment with high-dose steroids is recommended and patients should be referred urgently for temporal artery biopsy, which needs to be done within two weeks of starting treatment.

Commencing high-dose steroid treatment can prevent visual loss in a large majority of patients with GCA. However, once any visual loss has occurred, such treatment may prevent further deterioration but it is very unlikely that established visual loss could be reversed.

How common is giant cell arteritis?

GCA mainly affects people over the age of 60 and rarely affects people aged under 50, while women are more commonly affected than men. Statistics in the UK suggest a full-time GP may expect to see one new case every one to two years.

In practice, the condition should be suspected in anyone over the age of 50 with headache, scalp tenderness, temporary vision symptoms, or unexplained facial pain. Examination may show no abnormalities, but palpation of the temporal artery is often abnormal.

Headache and scalp tenderness are not essential to a diagnosis of GCA and their absence cannot be taken as an indication that the patient does not have the condition.

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