What is PMR and GCA?
Polymyalgia Rheumatica or PMR is a rheumatic condition which attacks twice as many females as males aged 55 and over. The average age of onset is round about 70 years.
It affects 1 in 2000 people. It can start slowly with flu like symptoms and it can start so suddenly that one morning you find you cannot get out of bed because you are so stiff and sore.
Diagnosis is difficult because other conditions, e.g. rheumatoid arthritis, have similar symptoms. The Doctor makes his diagnosis on the basis of a blood test and other factors. Early morning stiffness which eases as the day progresses is a significant factor along with severe pain in the shoulders, thighs and pelvic area. There is no known cause, no known cure and very little research is being done.
The most common treatment is initially a fairly high dose of Prednisolone, a steroid, which is reduced as quickly as possible because of the danger of osteoporosis if taken over long periods of time, along with treatment to avoid osteoporosis. The steroid treatment usually reduces the pain and stiffness quite quickly and often miraculously. What it is not so good at is alleviating the unreasonable fatigue and pain that continues for some people. In the case of most people the condition burns itself out after 2 - 2½ years. It can recur.
Giant Cell Arteritis (Temporal Arteritis)
Written in Layman’s terms by a Patient to help you understand GCA/TA. It has been checked by a medically qualified person to make sure it is factual within the current knowledge and understanding of GCA/TA.
It is hoped that with Research, the cause and a cure can be found in the future.
Giant Cell Arteritis (GCA/TA) and whom does it affect?
Giant cell arteritis 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. The artery most 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'. If left untreated it can cause serious sight loss.
Giant Cell Arteritis is sometimes related to other problems. Most commonly polymyalgia rheumatica, (PMR) a condition which causes pain in the shoulders and hip joints. People with PMR can sometimes develop Giant Cell Arteritis. However, Giant Cell Arteritis can occur on its own without any other medical conditions.
Symptoms or How do I know that I have Giant Cell Arteritis?
The main early symptoms of giant cell arteritis are headache and tenderness over the sides of the forehead. People with giant cell arteritis need urgent treatment with steroids. Early treatment will usually prevent serious complications such as eye problems and blindness.
- A Headache is the common symptom. This may develop suddenly, or it can come on gradually over several days or weeks. The headache can be one sided, or on both sides, mainly towards the front and sides of the head.
- Sometimes the temporal artery swells and can be seen around your eye, this too is tender to touch. Tenderness of the scalp over the temporal arteries is common. You may be able to feel one or both of the inflamed arteries under the skin.
- Other symptoms may occur and include: pain in the jaw or tongue, particularly when you chew or talk Often people are able to chew for a while but eventually the pain, becomes so bad they have to stop. If this happens you should be seek medical attention at either your GP or hospital as soon as possible.
- Problems with vision such as blurred vision or sudden loss of vision for a short time. These occur if the arteries going to these parts of the body are inflamed, become narrowed, and reduce the blood supply to these areas.
There are other symptoms such as fever, weight loss and generally feeling unwell.
Treatment for Giant Cell Arteritis/Temporal Arteritis
If giant cell arteritis is suspected, treatment is usually started straight away. The main aim is to reduce the risk of possible complications. The second aim is to relieve the headache and any other symptoms. A steroid medicine such as prednisolone is the usual treatment. Steroids work by reducing inflammation. After starting treatment, symptoms usually ease quickly, within a few days.
A high dose of steroid is started at first, usually about 40-60 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 1mg per day and a slow reduction programme will be worked out.
In some people the condition goes away after 2-3 years, so the 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.
Tests - Do I need any tests?
The ESR (erythrocyte sedimentation rate) and CRP (C reactive protein) blood tests are non-specific markers of inflammation. One or both of these markers is usually raised in GCA but in rare cases both can be normal. These blood tests can also be raised in other diseases and so a diagnosis of GCA cannot be based solely on a high ESR and/ or CRP - there must be other symptoms or signs of the disease or other abnormal test results to make a diagnosis of GCA.
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 giant cell arteritis a doctor can see the inflammation and abnormal giant cells in the sample of the artery wall. As well as taking a history from you about how the condition is affecting you, the doctor may well perform a number of other tests.
You should also be referred to the hospital ophthalmologist. And, if not it is a good idea to visit an optician to obtain a 'base line' of the state of your eyes for future use.
What are the possible complications of giant cell arteritis/temporal arteritis?
Complications are much less likely to occur if treatment is started soon after symptoms begin.
Possible complications of untreated giant cell arteritis include the following:.
- Vision Loss (blindness either temporary or permanent. The temporal artery provides blood to the optic nerve. These are the small arteries going to the eye. If this happens then the optic nerve is starved of blood and that artery becomes blocked it can cause permanent serious problems, even blindness.
Note: Arteries are blood vessels which take fresh blood to all the parts of the body. With Giant Cell Arteritis the medium-sized blood vessels become swollen. The most common artery to swell is the temporal artery.
- Make sure you understand that if any new visual disturbance occurs you must seek urgent medical advice if your GP is not available go to the nearest accident and emergency admission hospital and explain the situation.
Luckily Giant Cell Arteritis can be treated but there is currently no cure!
If you go to your GP with a new headache in the front part of your head along with some of the other symptoms mentioned, they may well send you to the hospital. A Rheumatologist or Ophthalmologist (eye specialist) can then confirm a diagnosis and prescribe a course of treatment that should prevent sight loss.
Giant Cell Arteritis caught early it can be treated with steroid tablets. Steroids help to reduce swelling, this mean that the swelling in the arteries can be reduced. The blood can then flow freely to the optic nerve again. This prevents the loss of vision.
Steroid treatment is started at a high dose and is reviewed closely. Over a period of time, sometimes a long period, the dose of the steroids can be reduced so that eventually you can stop taking them or control the disease on a very low dose.
Prednisolone is the most commonly used co-costeroid. These are known as 'steroids'.
This type of steroid is not the same as 'anabolic steroids' which are used in a completely different way.
There are also other medications, such as Methotextrate, but as steroids have been used since about 1949 and the side effects are well documented, at present Prednisolone is the preferred initial treatment.
Your symptoms of giant cell arteritis (GCA/TA ) should be eased with steroids!.
The plan will be to slowly reduce the dose to the lowest that keeps symptoms away. However, if you have a relapse, the dose will be increased.
Usage of Steroids:
- Do not stop taking steroid tablets suddenly and always carry a Blue Steroid Card or wear a Medi Tag (either a bracelet or necklace).
- Remember to tell any Medical Person who does not know you that you are on Steroids, the current dosage and for how long. 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.
- 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.
- Try to ensure that your steroids are ‘enteric coated. This reduces the risk of developing ulcers. Unfortunately 1mg steroids are not currently ‘enteric coated. Discuss with your Doctor to see is a gastro inhibitor is necessary.
- If buying ‘over the counter medications’, always check with the Pharmacist/ Chemist that they are compatible with Prednisolone. This also applies to alternative medication.
Possible Side Effects
these do not affect everybody and it is rare to have them all)
Keep the leaflet issued with your prescription and read it occasionally to refresh your memory.
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:
- Osteoporosis ('thinning of the bones') measures can be taken to protect against this if you have a high risk, bone protection tablets, DEXA scans, particularly necessary if you have had the menopause and/or are over 60. Your Doctor will advise.
- Weight Gain.
- Increased chance of infections; in particular, you should see a doctor urgently if you come into contact with chickenpox or shingles and you have not had chickenpox in the past.
- Increased blood pressure, have it checked regularly!
- High blood sugar, which may mean extra treatment if you have diabetes.
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.
Why Does Giant Cell Arteritis lead to blindness?
Giant Cell Arteritis is a condition that can seriously affect vision!
Sometimes if the symptoms of Giant Cell Arteritis are missed then people can suddenly lose sight in one eye. If this happens most people go straight to casualty since it is obvious that something is wrong. There they will see an ophthalmologist who will prescribed high dose of steroids usually intravenously (through a drip) this means you have to stay in hospital.
The high dose steroids are an attempt to bring down the swelling in the hope that re-establishing the blood supply quickly can bring sight back. Usually however this sight loss is permanent and the steroids are being used to stop the Giant Cell Arteritis affecting the sight in the other eye. . Only in about 25 per cent of cases will GCA cause the sight loss described if left untreated.
It is better not to take any risk. Any new pain on chewing, persistent headache, symptoms such as the swelling of the artery or pain when touching the temples should be reported to your GP or if out of hours or not available go to the nearest A&E Hospital Department. It is an Emergency.
This means that you can get the steroid treatment promptly and not risk the loss of your sight.
Research and Hope
There is a team led by Professor Bhaskar Dasgupta at Southend Hospital which is beginning some research. Along with the British Society for Rheumatology they are bringing out new guidelines for the diagnosis and treatment of PMR.
There is also an independent research unit in Arizona under the auspices of the National Medical Research Foundation.
Dr Sarah Mackie of Leeds University is undertaking a 3 year project, funded by Help the Aged, researching GCA. Marijke Foundation (Stichting Marijke MHKO Fonds) located in the Netherlands is concentrating on looking for Cause and Cure for GCA.
Reasons for lack of urgency in research so far:
- Few of us are still in the workforce, although this is changing.
- The conditions are not life-threatening.
- We are a generation brought up not to make a fuss, or even discuss illness - but that is changing.
You Are Not Alone!
There are quite a few of us out there and Support Groups are located throughout the UK.
Family and Friends will, in all probability not have heard of either PMR or GCA and as you will look OK, they will be bewildered and worried. The Support Groups welcome you and family, friends or carers are also most welcome.
You will meet people who are in the same position as you and also some with more experience. It is good to talk.