Temporal arteritis is a systemic, autoimmune, rheumatic condition in which the temporal arteries, which supply blood to the head and brain, become inflamed or damaged. Also known as cranial arteritis or giant cell arteritis, this condition is a type of vasculitis.

According to a review of research published in 2021, the overall incidence of the condition is about 10 cases per 100,000 people in individuals over 50 years old.

It is primarily a disease of older individuals, and the condition seems to have the highest incidence in northern European and Scandinavian countries. According to the American College of Rheumatology, women are also more likely than men to have temporal arteritis.

Although it usually occurs in the temporal arteries and other blood vessels in the head, it can also affect other medium and large blood vessels such as the aorta and its branches. People who have this condition are at risk of serious complications.

One of the most concerning complications is irreversible sudden blindness due to lack of blood flow to the ophthalmic artery or anywhere along the optic pathway.

lack of blood flow to the part of the eye called the optic nerve. The condition has an annual mortality rate of about 20 deaths per 1,000, per the 2021 research review previously referenced.

However, seeking immediate medical attention and treatment can reduce the risk of developing these complications. If you think that you may have temporal arteritis, you should see a doctor as soon as possible.

The symptoms of temporal arteritis can include:

  • double vision
  • Sudden, painless visual disturbance including temporary or permanent loss of vision in one eye or rarely both eyes
  • new headache or change from baseline headache
  • fatigue
  • weakness
  • loss of appetite
  • jaw pain that occurs with chewing or talking
  • cough
  • tongue pain,
  • sore throat or hoarse voice
  • numbness, tingling, weakness, or coolness of the arms or legs
  • fever
  • unintentional weight loss
  • shoulder pain, hip pain, and stiffness
  • tenderness in the scalp and temple areas often noticed after brushing your hair

These symptoms can also occur due to other conditions, and symptoms can vary in intensity, duration, and quality.

You should call your doctor anytime you’re worried about any symptoms you’re experiencing. They may refer you to a rheumatologist to get a thorough examination, the right diagnosis, and information on the best course of treatment.

The main treatment goal is to prevent permanent visual loss and to suppress inflammation of the blood vessels that can cause tissue damage.

If a doctor suspects you have temporal arteritis, treatment should begin immediately, even if test results haven’t yet confirmed the diagnosis.

Corticosteroids

The main treatment for temporal arteritis is corticosteroids. Even if the diagnosis is only suspected and the test results are still pending, your doctor may still prescribe oral corticosteroids immediately.

Prednisone is the most common corticosteroid used and has been proven to prevent visual loss. The response to prednisone is typically dramatic. Blood markers for inflammation usually improve within 2-4 weeks.

If there is no visual loss, the initial dose will typically be high, equating to about 1 milligram (mg)/kilogram (kg), usually about 40 to 60 mg a day.

If there is a visual loss or a high suspicion of impending visual loss, the dose may be high, and sometimes intravenous doses of high-dose steroids may be considered. The response to steroids is typically dramatic. Often, relief is seen within a few days. Your doctor will taper the corticosteroids slowly over the next 4 to 6 months.

Most people with temporal arteritis continue taking corticosteroids for at least a year. Some may have to take a permanent low dose.

Corticosteroid side effects

While you’re undergoing corticosteroid therapy, it’s important that you schedule regular checkups with your doctor. They’ll need to monitor your progress as well as the way that your body is handling medical treatment.

Your doctor will monitor your inflammation via lab testing every 2-4 weeks in the first six months. Afterward, testing will depend on your individual symptoms. This is because prolonged use of corticosteroids can have severe effects on your bones and other metabolic functions.

It’s important to know that corticosteroids can increase your risk of developing other medical conditions, such as:

Other potential side effects include:

Talk with your doctor about ways to minimize these side effects.

Other treatments and helpful lifestyle changes

Although corticosteroids are the most common and effective treatments for temporal arteritis, in certain cases, doctors may consider other treatments as add-on therapy to help taper off the use of corticosteroids such as prednisone. This add-on therapy is referred to as steroid-sparing agent or therapy.

This may be particularly important if you develop significant side effects from corticosteroids or if you are at high risk of developing side effects as is the case with having diabetes or osteoporosis.

Steroid-sparing agents include certain chemotherapy such as Methotrexate and immunosuppressant medications such as Tocilzumab (Actemra), which is FDA approved for the treatment of temporal arteritis. In some cases, a daily dose of aspirin in addition to other treatments may help reduce the rate of vision loss and prevent stroke.

The following measures may also help reduce the severity of some symptoms, as well as the severity of long-term side effects from corticosteroid use:

You’ll still need to see your doctor for checkups once you’ve finished your course of treatment. This is because temporal arteritis can recur. Also, be sure to talk with your doctor before making any changes to your treatment plan.

Temporal arteritis has no definitive cause, but researchers speculate that a viral or bacterial infection may be the trigger in many cases.

Some of the suspected viruses and bacteria that may be triggers include:

  • varicella-zoster virus (VZV), which causes chickenpox and shingles
  • herpes simplex virus
  • Epstein–Barr virus
  • parvovirus B19
  • chlamydia pneumoniae
  • mycoplasma pneumoniae

However, there is no evidence to definitively prove or disprove this. Temporal arteritis, once it occurs, appears to be autoimmune in nature. This means that the body’s own immune cells attack healthy tissue in the artery walls.

A rheumatologist is most likely to suspect temporal arteritis if a person is over 50 years old and experiences a local headache that has not existed before.

Your doctor will perform a physical exam and look at your head to determine whether there’s any tenderness. They’ll pay special attention to the arteries at your temples. The examination may show a decrease in pulsation or increased thickening of the arteries at your temples.

Your doctor will then make the diagnosis based on a number of factors including signs and symptoms, and blood test or tissue biopsy results.

Additional indicators are the erythrocyte sedimentation rate (ESR) test and the C-reactive protein (CRP) test. These tests are usually very elevated with giant cell arteritis.

ESR measures how quickly your red blood cells collect at the bottom of a test tube over 1 hour. If the rate of collection is greater than or equal to 50 millimeters (mm) per hour, this may indicate you have the condition. However, these markers are nonspecific and can be elevated in other conditions such as kidney disease, anemia, age, malignancy, and other conditions.

The CRP test is used to measure your inflammation during the taper of corticosteroids to monitor for any relapses This is a more specific marker for inflammation but is also nonspecific. Elevations of both markers improve the specificity of the diagnosis. If both markers are normal, the diagnosis is less likely to be giant cell arteritis.

Typically, you will also need to have a biopsy of the temporal artery to aid in the diagnosis. The doctor will remove a sample of the temporal artery either from above or in front of your ear. The procedure is usually low risk and short in duration. When examined under a microscope, a positive sample can show inflammation of the artery as well as changes within the artery itself.

The doctor may see characteristic “giant cells” in the biopsy results, but these cells are not necessary for diagnosis and can be absent in about half of all cases.

A biopsy is an important diagnostic tool, and every effort should be made to obtain a biopsy. If a biopsy is positive and shows characteristic cells, this is confirmatory, and the diagnosis is certain.

There can, however, be false-negative results if the temporal artery has both areas of normal segments and areas of inflammation.

If a doctor suspects you have temporal arteritis based on your risk factors and other supporting data, even if the biopsy is negative, they may choose to make the diagnosis and continue your treatment for temporal arteritis.

You can get a biopsy as part of an outpatient procedure using local anesthesia. An ultrasound may also provide additional information about whether have temporal arteritis.

If you have symptoms or a diagnosis of temporal arteritis, it’s important to get treatment immediately to prevent serious complications. Visual loss or blindness is the most serious complication. Other complications can include:

  • inflammation and damage to other blood vessels in the body
  • development of aneurysms, including aortic aneurysms, and a more rarely occurring aortic dissection
  • eye muscle weakness
  • stroke

An aortic aneurysm, if ruptured, can lead to massive internal bleeding. Survival is not impacted with giant cell arteritis unless complications of the aorta occur. Talk with your doctor about ways to minimize any complications from the condition.

Your outlook for temporal arteritis will depend on how quickly you’re diagnosed and start treatment. Untreated temporal arteritis can cause serious damage to the blood vessels in your body, and in some cases, it can be life-threatening.

Call your doctor if you notice any symptoms. This will make it more likely that you’ll be diagnosed with a condition when it’s in the early stages.

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