Temporal Arteritis, (also called Giant Cell Arteritis) is a cause of new onset headache in older people.
It is rare under the age of 50 years.
The headache itself is not necessarily that painful, but the consequences of untreated arteritis are severe.
Irreversible blindness is the dreaded complication when arteritis is undetected. Unfortunately this can still occur in up to 20% of people with this condition.
Even if treated, there is still a risk of visual loss, but early treatment with corticosteroids like prednisolone can reduce this risk.
For that reason anyone over the age of 50 years who develops a new headache should always have their ESR measured (ESR = Erythrocyte Sedimentation Rate) measured, and consideration given to this diagnosis.
The headache is usually located on the side of the head.
About 50% of people will have headache as the initial symptom but by the time of diagnosis this is over 90%.
The main arteries affected are branches of the superficial temporal artery. This artery runs just in front of the ear and supplies the front and side of the scalp of your head.
The other artery affected is the occipital artery, which supplies the back of the head. I have had patients in whom I have confused Temporal/Giant Cell Arteritis with cervicogenic headache, as their main headache was a severe occipital pain which was worse with lying down.
One study of older people (Solomon 1987) found that the temples were the site of pain in only 25%, and 28% had the occiput as the site of pain.
Please be vigilant.
This is not a diagnosis to miss and should always be in the forefront of your mind if you have a new onset headache over 50 years.
People who start to get visual problems may present with an episode of transient visual loss which could initially be confused for a more common problem of Transient Ischaemic Attack (TIA).
Arteritis should be suspected as a cause of a TIA if the ESR test is elevated or headache or general malaise is also present.
If visual loss starts to occur, this can feel like a cloud coming over the visual field which persists. Visual acuity may fall rapidly, and complete blindness could ensue if prednisolone is not administered urgently.
There are other symptoms like peripheral neuropathy (e.g. ulnar neuropathy), or swelling of ankles.
Other arteries, such as those to the lower limbs can be affected - I have seen a case which presented with leg muscle cramps provoked by walking (intermittent claudication).
Fever can also occur - as high as 39 degrees celcius, and arteritis is a cause of "Pyrexia of Unknown Origin".
The current recommended diagnostic criteria (American College of Rheumatology) are:
- Age >50years
- New onset headache
- ESR >50mm
- Abnormal temporal artery e.g. loss of pulsation or tenderness
- A positive temporal artery biopsy showing giant cells
If you have 3 of these 5 criteria there is a 90% chance you do in fact have temporal arteritis.
The main issue, like all severe headache disorders, is to suspect the diagnosis.
If you suspect the diagnosis, make sure you palpate the cranial arteries for tenderness and pulsatility.
Make sure you record visual acuity and record a visual field. Then check the ESR, or C-reactive protein if ESR.
If you remain convinced that Temporal Arteritis is a possibility, start prednisolone and arrange a temporal artery biopsy urgently (ideally within 2 weeks).
If you suspect the diagnosis and your patient already has impaired vision, you could use mega-dose (500mg to 1000mg infusion) Intravenous Methylprednisolone while waiting for results of biopsy.
Remember to be careful in using steroids in people with diabetes or previous cardiac disease.
In studies designed to predict the diagnosis the following seem to be more strongly associated with a positive biopsy (Niederkohr 2005; Hayreh 1997):
- Cramping in jaw muscles on chewing - <em>jaw claudication</em> (4 to 9 times chance of positive biopsy)
- ESR greater than 107mm (2.7 times increase chance of positive biopsy)
- Pain in the temporal artery (2.3 times chance of positive biopsy)
- Any visual symptom (1.3 times chance of positive biopsy)
- Weight Loss (1.3 times chance of positive biopsy)
- Headache ( 1.2 times increased chance of positive biopsy)
Headache is the weakest association with a positive biopsy. This is because there are so many different types of headache disorder that might be confused with temporal arteritis.
Temporal Artery Biopsy
The segment of artery should be at least 3cm long, and taken, if possible from the more symptomatic side.
Biopsies can remain positive for up to 6 weeks after steroid treatment, so a biopsy should not be a reason to defer steroid treatment.
In studies of ultrasound for temporal arteritis the changes in the arteries persisted for over 2 weeks after starting steroids.
Some hospitals will do an immediate frozen section on a temporal artery biopsy sample, and if the frozen section is negative a further biopsy, from the other side or other symptomatic artery could be obtained.
The main problem in interpreting the biopsy is that the abnormal areas can be found in patches (this is in common with lots of neuroinflammatory conditions), so the biopsy needs to be carefully examined under the microscope before concluding it is negative.
Ultrasound has been reported as a useful test for giant cell/temporal arteritis. It may even help guide biopsy to more affected areas.
The New England Journal of Medicine reported on this in 1997, and proposed that ultrasound could replace biopsy.
However, biopsy remains the preferred option. If I was suspected of having arteritis, I would want to be as certain as possible before committing myself to long term steroid treatment.
ESR and CRP tests
The Erythrocyte Sedimentation Rate (ESR) is reported in millimetres (this is the distance the red cells fall in a vertical tube designed for doing the test).
The Erythrocyte Sedimentation Rate (ESR) is a blood test that looks at how quickly blood separates into cells and serum.
In temporal arteritis there are a lot of extra proteins in the blood, so this separation takes place rapidly.
The average ESR in temporal arteritis is 85mm. 97% of people with a diagnosis of arteritis will have an ESR greater than 50mm.
A normal ESR is a sensitive test - if it is negative it rules out the condition in most people.
I would only chase the diagnosis if there was temporal artery change or jaw claudication or unexplained visual impairment in an older person.
The C-reactive Protein (CRP) is, on average greater than 25mg/l in active arteritis, and many laboratories no longer use the ESR but will recommend the CRP instead. Interleukin-6 is also being tested as a marker for the disease.
What is the prognosis (outlook) from temporal arteritis?
Within a few days of starting treatment, most people start to feel an awful lot better.
A widely quoted medical saying is that failure to improve in a day or two should make you question the diagnosis. A headache may linger for about a month.
It would be normal to require a year or more of steroid treatment for temporal arteritis. In fact the average in one study was 22 months.
After steroids are withdrawn, there is a 50% chance that symptoms will return within a month of removal of steroids.
This means that repeated course of steroids are required and about 40% of people, 5 years from their first diagnosis, will still need steroids.
At 9 years the figure was 25%. This is the price to be paid for preserving eyesight which is the main complication of untreated arteritis.
If there are no references listed, please notify me, as this box should contain a list of information sources, or a link to a 'Systematic Review' of the topic discussed on this page.
BSR and BHPR Guidelines for the management of giant cell arteritis
Bhaskar Dasgupta et al Rheumatology 2009. doi:10.1093/rheumatology/keq039b
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