Giant cell arteritis (GCA)


Giant cell arteritis (GCA) is a form of vasculitis. Vasculitis causes an inflammation of the blood vessels that typically has some immune system-related cause. GCA can cause inflammation, swelling and thickening of the small artery under the skin called the temporal artery, but other blood vessels can also become inflamed. In GCA, the vessels of the head are most involved (especially the temporal arteries, located on each side of the head). There is also evidence of giant cell arteritis when inflammations in (middle) large arteries occur in the body, such as at the aorta, branches of the aorta or other arteries towards the head or brain.

The complaints and abnormalities that occur with GCA are caused by the inflammation causing a narrowing, resulting in a poor blood flow, or closing of the artery. This causes the body part or organ that is supplied with blood through that artery to receive less blood. This can lead to pain, but also to tissue death. Signs and symptoms may include a new, persisting throbbing headache on one side of the head or the back of the head, tenderness of the scalp, various symptoms that feel like the flu, unexplained fatigue (tiredness) or fevers and/or problems with eyesight. If GCA affects the blood flow to the eye, loss of vision can occur. Prompt detection and treatment of GCA can prevent loss of vision. A new, persisting headache is a common symptom of GCA. Early treatment is important.

Giant cell arteritis (GCA) may occur together with a joint pain condition called polymyalgia rheumatica. The cause of GCA is still being studied, but an abnormal immune response has been implicated. Several genetic and environmental factors may increase a person’s risk to develop GCA.

Giant cell arteritis (GCA) occurs only in older adults, mainly those over the age of 50. The disease is more common in women than in men, especially in women over 50 years of age.

Cause of Giant cell arteritis

The cause of the occurrence of giant cell arteritis (GCA) is not clear. Giant cell arteritis (GCA) is a form of vasculitis. Vasculitis causes an inflammation of the blood vessels that typically has some immune system-related cause. GCA can cause inflammation, swelling and thickening of the small artery under the skin called the temporal artery, but other blood vessels can also become inflamed. In GCA the vessels most often involved, are the arteries of the scalp and head, especially the arteries over the temples, which is why GCA is also called ‘temporal arteritis‘. The complaints sometimes occur quite suddenly, but usually GCA gradually develops. Giant cell arteritis is not hereditary or contagious.

The complaints and abnormalities that occur with GCA are caused by the inflammation causing a narrowing, resulting in a poor blood flow, or closing of the artery. This causes the body part or organ that is supplied with blood through that artery to receive less blood. This can lead to pain, but also to tissue death. Because different arteries can be inflamed, the disease can lead to various complaints and abnormalities.

The disease is more common in women than in men, especially in women over 50 years of age and occurs only in adults usually over age 50.

Giant cell arteritis (GCA) can overlap with the disease polymyalgia rheumatica (PMR), also called muscle rheumatism. Giant cell arteritis occurs in approximately 5% to 15% of people with the diagnosis polymyalgia rheumatica. About 50% of patients with GCA have symptoms of PMR. The two conditions may occur at the same time or on their own and affect both the same types of patients.

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Complaints in Giant cell arteritis

The complaints and abnormalities that occur with giant cell arteritis (GCA) are caused by the inflammation causing a narrowing or closing of the artery. The most common felt symptom of GCA is a new persistent throbbing headache, usually around the temples, but the headache due to GCA can occur anywhere, including the front, top and back of the skull. Almost as common are symptoms such as fatigue, loss of appetite, weight loss, a flu-like feeling, possibly pain in the jaw with chewing food or sometimes just an unexplained fever. Less common symptoms include pains in the face, tongue, or throat. When the giant cell arteritis spreads to the blood supply of the eye, your eyesight can be affected. Problems with vision can include temporary blurring, double vision or blindness. The permanent loss of vision can occur suddenly, but proper treatment can prevent this complication.

Giant cell arteritis may coexist with polymyalgia rheumatica (PMR), also called muscle rheumatism. A typical complaint of muscle rheumatism is long-term (at least four weeks) muscle pain and stiffness in the neck, shoulders and/or pelvis. If you have headaches on one side of your head in addition to other symptoms of polymyalgia rheumatica (PMR), you may have giant cell arteritis. Often only the blood vessel on one side of the head is inflamed, but you can also get an inflammation of the blood vessels on both sides of the head. It is important that patients who have active or inactive PMR report any symptoms of a new headache, changes in vision or jaw pain right away to the doctor.

The symptoms with CGA occur mainly in the head and neck area:
severe headaches at the height of your sleep, usually on one side of the head or sometimes on both sides of your head.
sensitive and swollen scalp during sleep (hair combing can hurt).
(muscle) pain and stiffness in the back of the head, skull and neck.
pain in your jaws during chewing.
fatigue, nausea, lethargy, weight loss, muscle pain, stiffness, fever.
night sweating.
less vision, double vision, blurred vision or spots on the eyes. If you suddenly start to see worse, get eye complaints or if there is sudden or rapidly increasing visual impairment, you should contact your doctor or ophthalmologist immediately. If you are not treated on time and the artery is blocked to the optic nerve, you can blind one eye. If your original eye complaints come back after your treatment, it is important to bring forward the control appointment with your doctor.

Diagnosis in giant cell arteritis

Your doctor will base your diagnosis on a combination of the medical history, his findings and additional test results.

At the first visit, the doctor asks you a number of questions to get a good picture of your condition. You can think of the following questions:
– When did your complaints start?
– Can you describe your complaints?
– How are your working conditions?
– What medication do you use?
– Do rheumatic diseases occur in your family?
– What is your medical history?
– Are you under the treatment of other specialists and for what?
If you have complaints, the doctor will perform physical examination and blood tests. This is necessary to confirm the diagnosis.

Blood tests. There is no simple blood test or noninvasive way to confirm the diagnosis of giant cell arteritis (GCA). However, the doctor can have your erythrocyte blood sedimentation or the amount of C-reactive protein (CRP) measured in your blood.
Sedimentation speed. Blood tests also measure the degree of inflammation through the sedimentation rate of the red blood cells. An erythrocyte sedimentation rate (ESR) test is sometimes called a sedimentation rate test or sed rate test. The erythrocyte sedimentation rate (or sed rate) is a blood test that measures inflammation by checking to see how the rate at which red blood cells (erythrocytes) sediment (or fall) within an hour. If these two values are (strongly) increased, it is a sign that you have an inflammation in your body. Because other diseases can cause high sedimentation rates, doctors cannot rely on this finding alone as proof of GCA. This blood test doesn’t diagnose one specific condition. Instead, it helps your doctor determine whether you’re experiencing inflammation. This can be the result of infections (bacteria, viruses), tumors and autoimmune diseases (arthritis).
C-reactive protein (CRP) is a substance produced by the liver in response to inflammation. A high level of CRP in the blood is a marker of inflammation. It can be caused by a wide variety of conditions from infection to cancer.
Ultrasound CT scan. In some cases, an ultrasound of the temporal arteries on the side of head can be done. If positive this can suggest an active giant cell arteritis (GCA). A negative test, however, does not rule out GCA.
Biopsy. With a biopsy (a surgical removal), your doctor removes a small piece of temporal artery to study it under a microscope for signs of inflammation. This biopsy is an outpatient procedure, done under local anesthesia. It leaves just a small scar that usually cannot be seen at the hairline in front of the ear. If the piece of tissue shows a venous inflammation, then the diagnosis is giant cell arteritis. Sometimes the inflammation can not be seen in the biopsy. If there is doubt about the diagnosis based on the first biopsy, your doctor may do a second biopsy of the temporal artery on the other side of your head. Yet if nothing is found you can still have inflammation, because the inflammation of the artery can be very local and only a few millimeters away. It is therefore possible that the doctor does not find any inflammatory material in the tested piece, while you do have CGA.
PET scan. With a PET scan, a study that uses light radioactivity, inflammation in the body can be visualized in the larger arteries.

Your doctor will make the final diagnosis of giant cell arteritis (CGA) based on the following information:
• Your age (are you 50 or older)?
• Does it hurt when the doctor presses your sleep?
• Do you have an increased blood sedimentation?
• The type of headache you have?
• Do you experience a throbbing in the artery?
• Is a vein inflammation visible in the biopsy under the microscope?
• What are the results of the Ultrasound CT scan or PET scan?

Treatment Giant cell arteritis

Drug treatment
Drugs without a prescription
• For the pain you can get a simple painkiller with the active ingredient paracetamol without a prescription. Paracetamol helps against pain and fever, does not cause stomach problems, usually does not cause side effects and can be easily combined with other medications.
• An NSAID, an anti-inflammatory analgesic in a lower dose. The abbreviation NSAID stands for Non-Steroidal Anti-Inflammatory Drugs, such as diclofenac, naproxen and ibuprofen. These painkillers inhibit inflammation.

Do you have physical complaints? Always go to your doctor or specialist for a proper diagnosis and proper treatment.

Drugs on prescription
This is provided by your doctor or rheumatologist or internist.
The treatment for giant cell arteritis (GCA) should begin as soon as possible because of the risk of loss of vision. If your doctor strongly suspects you have GCA, the treatment can start before you get the results of a temporal artery biopsy.
• An NSAID, an anti-inflammatory analgesic. The abbreviation NSAID stands for Non-Steroidal Anti-Inflammatory Drugs. These are medicines that inhibit inflammation, but do not contain corticosteroids (steroids). These anti-inflammatory painkillers are all effective against pain, inflammation, stiffness and fever, but do not prevent joint damage. Your doctor will prescribe these prescription drugs with a higher dose of the active substance.
A corticosteroid (an artificial adrenal cortex hormone) is a drug that resembles the natural hormone that the body produces in the adrenal cortex. They are anti-inflammatory drugs that suppress the immune response in the body and inflammation. Your doctor will prescribe these prescription drugs with a higher dose of the active substance. Unlike the treatment for polymyalgia rheumatica (PMR), which requires only low-dose corticosteroids (also called glucocorticoids). Glucocorticoids reduce inflammation throughout your body. They do this by interfering with the way certain chemicals in your body cause inflammation. The treatment of GCA usually involves high doses of the corticosteroid prednisone (Deltasone, Orasone and so on). Headaches and other symptoms quickly decrease with treatment and the sedimentation rate declines to a normal range.
The high dose of corticosteroids usually continues for a month and then the dose is slowly decreased. The speed in which your doctor lowers the dose may change if you have recurring symptoms of GCA or large increases in the sedimentation rate. In most cases though, the prednisone dose can be reduced over a few months. Patients are usually tapered off this medicine by one to two years. Giant cell arteritis (GCA) rarely returns after treatment.
• A conventional synthetic DMARD (Disease-Modifying Anti Rheumatic Drug), for example methotrexate, sulfasalazine (Azulfidine), gold, hydroxychloroquine, azathioprine, leflunomide and ciclosporin may be prescribed. A DMARD has different mechanisms of action and suppresses joint inflammations in a number of forms of arthritis. When using a DMARD at an early stage of your condition, your joints will be less damaged by the inflammations. These drugs not only relieve symptoms but also slow down progression of the joint damage. Often, DMARDs are prescribed along with non-steroidal anti-inflammatory drugs, such as NSAIDs and/or low-dose corticosteroids, to lower swelling and pain. A DMARD can cure the symptoms of giant cell arteritis (CGA).
• A biologic DMARD (TNF alpha inhibitors, Interleukin inhibitors, B cell inhibitors and T cell inhibitors). A biologic response modifier is a drug that can affect your immune system. If you are healthy, the immune system itself produces enough antibodies to defend itself against pathogens (viruses and bacteria). This balance is disrupted in people with chronic inflammatory disease. A biologic response modifier can restore that balance. The drugs can block the immune system chemical signals that lead to inflammation and joint/tissue damage. Most often, patients take these drugs with methotrexate, as the mix of medicines is more helpful. Tocilizumab (Actemra) is a biologic response modifier drug currently approved to treat adults with giant cell arteritis (GCA). Tocilizumab blocks the inflammatory protein IL-6. This improves joint pain and swelling from arthritis and other symptoms caused by inflammation.
• A biosimilar. A biosimilar drug is a drug that is developed to be highly similar and clinically equivalent to an existing biologic. A biosimilar drug contains a version of an active substance of an already approved biologic drug (the ‘reference drug’ or ‘originator drug’). Similarity to the reference biologic in terms of quality, structural characteristics, biological activity, safety and efficacy must be established, so that there are no clinically meaningful differences from the biologic in terms of quality, safety and efficacy. Research has shown that the biosimilar is just as effective as the original biologic inhibitor in the treatment of arthritis.
Biosimilar drugs are not the same as generic drugs, which contain simpler chemical structures and are identical, in terms of molecular structure, to their reference drugs.

Use of drugs and adherence

• It is important that you take the drugs as prescribed. In order to get your illness under control, it is very important that you take your drugs on a regular basis every day. If you do not, there is no good concentration of the drug in your blood and therefore the drug will work less well.
• Do you have trouble remembering when and how many of your drugs you should take on a daily basis and at what time of the day? Then buy a drug dispenser, in which you can sort your drugs per day.
• Do you have difficulty or objection to taking the drug prescribed to you, for example because of possible side-effects? Then that can lead to you no longer taking the drug regularly. Discuss this with your doctor!

Deterioration of your illness and/or complications
The doctor will look at:
• The dosage and amount of drugs you use.
• Switching to another drug in the same type or a completely new type of drug.
• Combining different drugs at the same time.

Your doctor can tell you
• whether the drug can work for you.
• how you can best use the drug.
• how much and how often you can use the drug.
• how you can best reduce the drug.

You should always tell your doctor or specialist
• if you use other drugs (bought or prescribed by another doctor).
• if you have another medical condition.
• whether you will be operated on soon.
• if you have previously been prescribed an anti-inflammatory analgesic that has caused side effects.
• whether you want to become pregnant or are pregnant.
• if you are breast-feeding.
This is important because your doctor or specialist has to make a careful choice between different drugs.

• All drugs may have side-effects when used, so ask your doctor or pharmacist what side-effects you can expect or read the leaflet.

Reduce or stop
• If you stop using a drug yourself or reduce the number of drugs yourself, your symptoms may worsen. Always consult your doctor or specialist first if you want to use less drugs or want to stop.

Alternative treatments

There are many types of alternative treatments. This refers to all treatments that fall outside normal scientific medical care. No scientific evidence has been provided for the operation of these treatments. The alternative treatments are also called ‘complementary‘ because they can supplement the regular medical treatment by your doctor.
Many people choose an alternative treatment in addition to their regular medical treatment. They hope that this helps extra against their complaints or better helps to deal with the complaints.

Can an alternative treatment be a replacement for your regular medical treatment?
No, if you opt for an alternative treatment method, it is always a supplement to your regular medical treatment. You should not stop your regular treatment, because otherwise you run unnecessary health risks. Always consult your attending doctor before you start an alternative treatment.

What alternative treatments are there for example?
There are many different alternative treatment methods available, which can also be combined. Some examples are:
• Chinese medicine (acupuncture, pressure point massage, nutrition and tai chi).
• homeopathy (products made from plants and minerals).
• Bowen therapy.
• Ayurveda.
• Bach flower treatment.
• shiatsu, foot-sole and classic massage.
• Touch for Health.
• Reiki.
• dietary supplements.

What do you have to pay attention to?
• Many alternative treatments have not shown that they actually work. If you choose to try alternative treatment, make sure that your complaints do not increase. Stop an alternative treatment as soon as your symptoms increase.
• Always consider first why you want to follow an alternative treatment.
• Prepare yourself by looking up and reading information about the alternative treatment. Consult with your attending doctor, because certain complaints may be reduced by some alternative treatment methods.
• Determine yourself in which alternative method you trust for the relief of your complaints and whether you want to start.

What can you do best when choosing an alternative treatment?
• Always consult your doctor about the alternative treatment method you want to follow.
• Consult with your attending doctor and with the alternative therapist if they want to discuss your treatment with each other.
• Choose an alternative therapist who has followed a recognized vocational training and is affiliated to a professional organization.
• Ask your alternative therapist in advance about the purpose, duration, costs and risks of the treatment. How much money you spend depends on which treatment you choose and how long it takes.
• Do not stop your regular medical treatment, as this may worsen your symptoms.
• Weigh during treatment whether you want to continue or stop if your symptoms get worse, if you do not notice any effect of the alternative treatment or if you get side-effects.

Why is your food important?

• Healthy nutrition is important to get the vitamins and minerals and other nutrients that your body needs.
• Obesity creates risks for your health and disease progression. In the case of arthritis and obesity, for example, obesity plays an important role in overburdening and osteoarthritis in the knees, hips and ankles. The pressure on your joints is then simply too great. Healthy eating and exercise (exercising a sport and active in the home and outdoors) can help to reduce or prevent excess weight.
• A healthy diet is always important and the dietitian can support you with the right dietary advice.

What is a healthy diet?
With a healthy diet your body gets the right amount of good nutrients, which it needs. You eat the quantities that your body needs. The dietitian can support you with the right nutritional advice.
The 5 courses to choose from are:
• Vegetables and fruit
• Bread, cereal products and potatoes
• Fish, legumes, meat, egg, nuts and dairy
• Lubrication and preparation fats
• Drinks.

Where can you get advice for a healthy diet?
• You can request advice from your doctor or a dietitian. The doctor can refer you to a dietitian. Some diets may possibly help against your symptoms. Consult with your doctor or dietitian if you want to try a certain diet. And make sure that you do not omit important foods from your daily diet.

What can you do with a painful or dry mouth?
Sometimes you can suffer from a dry or painful mouth for various reasons, also due to your illness. Tips:
• by chewing (for example piece of cucumber, sugar-free candy or chewing gum) and sucking (ice cube) the salivary glands are stimulated to produce saliva.
• good oral care is important: good tooth brushing, flossing and the use of mouthwash.
• rinse your mouth regularly, drink small amounts of water and use a mouth sprayer if necessary.
• let hot drinks cool down first.
• use ice or cold dishes, because the cold numbs the pain.
• do not use sharp herbs and spices, fruit juice, carbonated soft drinks, alcoholic beverages, very salty foods and sour food.
• food with hard crusts, nuts, bones and bones can cause injuries.
• use soup, gravy or sauce with the hot meal to make the food smoother.
• spreadable cheese, spreadable paté or salad, jam or honey on bread, porridge, drinking breakfast and custard, for example, swallows easier than dry spreads.
• if you need to use ground or liquid food, you can use a mixer to grind your meal with some extra moisture.

In some forms of arthritis or use of certain drugs intestinal complaints can occur. The intestinal complaints can arise from the influence of certain drugs on food intake. Conversely, the food you eat can have an effect on the way your drugs work.

Dietary supplements, fish fatty acids, glucosamine and vitamin D?
• Dietary supplements are available as pills, powders, drops, capsules or drinks and are intended as a supplement to inadequate daily nutrition. They contain vitamins, minerals or bio-active substances. These synthetic or isolated vitamins, minerals or bio-active substances have the same effect as the vitamins and minerals that are already naturally in your food and drink.
Many people choose an additional supplement, in addition to their daily diet. The users of dietary supplements indicate that they experience positive effects of the use of certain herbs, vitamins and minerals. The body absorbs the nutrients in pills more easily than the nutrients in food. But if you eat healthy and varied, you do not need additional nutritional supplements, because you already get enough nutrients, minerals and vitamins. Always report to your doctor and pharmacist that you are using dietary supplements.
• Fish fatty acids appear to have a mild anti-inflammatory effect with a high intake. Research has been done especially in rheumatoid arthritis. The advice is to eat oily fish twice a week.
• Glucosamine can act as a mild painkiller for osteoarthritis in the knee, but does not stop arthritis.
• Our body naturally makes vitamin D under the influence of outdoor sunlight. Vitamin D may have a beneficial effect in inflammatory arthritis, but that has not yet been proven sufficiently. People with Lupus erythematosus get vitamin D prescribed as they are not often outside, because sun exposure can lead to lupus flares and skin problems. Vitamine D is given with calcium tablets to ensure a good intake in the body, when you have osteoporosis or osteoarthritis.

Living with Giant cell arteritis

• If you are treated for a long time with a higher dose of a corticosteroid, this can cause bone loss and osteoporosis. So the doctor may want you to get a bone density test if you are using the drug for a long time. Some of the other side effects from a high-dose corticosteroid are jitteriness, poor sleep and weight gain. These can be unpleasant but are reversible. They get better as you take smaller doses. Muscle weakness, cataracts and skin bruising also can occur with corticosteroid use.
• The doctor can suggest you take supplements of calcium and vitamin D to protect against osteoporosis and the risk of fractures (broken bones). Your doctor can also prescribe you to take a prescription drug to protect your bones. These include the bisphosphonates: risedronate (Actonel), alendronate (Fosamax), ibandronate (Boniva), or zoledronic acid (Reclast).

Osmosis I Vasculitis (Giant cell, Takayasu, Polyarteritis nodosa, Buerger, Granulomatosis)

Source videos
Osmosis I Vasculitis (Giant cell, Takayasu, Polyarteritis nodosa, Buerger, Granulomatosis) I
Patient Education Library I Polymyalgia Rheumatica and Giant cell arteritis I

Accountability Giant cell arteritis
The information about giant cell arteritis is general.
Every situation is different, so if you have any questions or complaints, always consult your doctor, medical specialist or pharmacist.

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