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Giant Cell Arteritis (GCA)

Last updated May 28, 2018

Approved by: Krish Tangella MD, MBA, FCAP

DoveMed.com

Microscopic pathology image showing a normal temporal artery biopsy on an elastic stain performed for a Giant cell temporal arteritis.


What are the other Names for this Condition? (Also known as/Synonyms)

  • Cranial Arteritis (due to Giant Cell Arteritis)
  • Horton Disease
  • Temporal Arteritis (due to Giant Cell Arteritis)

What is Giant Cell Arteritis? (Definition/Background Information)

  • Giant Cell Arteritis (GCA) is a systemic autoimmune inflammatory disease that results in inflammation of the blood vessels. This inflammation results in destruction that causes blood vessels and arteries to occlude. The condition typically affects older women in their 70’s
  • GCA is an autoimmune disease, since it is characterized by the body’s immune system attacking its own blood vessels. Typically, the inflammatory cells target the temporal artery and various cranial arteries, including the vertebral, cerebral, ophthalmic, and retinal arteries. In some cases, it may also attack the aorta and subclavian arteries
  • Arteries and blood vessels are very important components of the circulation system; they carry blood and nutrients from the heart, to tissues in the body. When blood vessels and arteries become inflamed and occluded (obstructed), the blood flow is reduced. This means that the nutrient supply to the tissues is also lowered, causing damage to tissues that can be harmful to the body
  • Anti-inflammatory medication is the treatment of choice for Giant Cell Arteritis. The prognosis is usually good with early diagnosis and treatment

Who gets Giant Cell Arteritis? (Age and Sex Distribution)

  • Giant Cell Arteritis is most common in individuals over the age of 50 years. The median age of onset is 75 years
  • 75% of all GCA cases occur in females, implying that the female-male incidence ratio is 3:1
  • The disorder is more commonly seen in individuals of Caucasian descent; although, it can appear in all races and ethnicities

What are the Risk Factors for Giant Cell Arteritis? (Predisposing Factors)

Common risk factors of Giant Cell Arteritis include:

  • An advancing age - older adults are most commonly affected
  • Gender - females have a higher risk, than males
  • Individuals belonging to a Caucasian ethnicity
  • Polymyalgia rheumatica: A disorder causing pain and stiffness in the hip, shoulder, and neck; about 15% of individuals with polymyalgia rheumatica, suffer from GCA

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Giant Cell Arteritis? (Etiology)

  • The exact cause of Giant Cell Arteritis is largely unknown
  • However, it is known that the risk of developing GCA increases with an advancing age. Similarly, women have a higher risk, as well as individuals of Caucasian descent

What are the Signs and Symptoms of Giant Cell Arteritis?

Signs and symptoms of Giant Cell Arteritis vary widely, depending on which blood vessels are attacked by the immune system. However, the common signs and symptoms of GCA that may be present include:

  • Headache (can be generalized)
  • Scalp tenderness
  • Tiredness
  • Facial pain, jaw pain due to jaw movement
  • Flu-like symptoms with low-grade fever
  • Muscle pain (commonly felt in the neck, shoulder, and hip)
  • Swollen arteries, other blood vessels
  • Blurred vision, double vision, loss of vision
  • Loss of appetite, weight loss
  • Transient ischemic attack  (TIA), cerebral vascular accident (CVA)
  • Vertigo

How is Giant Cell Arteritis Diagnosed?

To diagnose Giant Cell Arteritis, a variety of tests may be performed:

  • Giant Cell Arteritis is first suspected during a physical examination, when a physician performs an evaluation of all body systems and collects a thorough medical history. Based on a combination of risk factors and symptoms, a diagnosis of GCA is made
  • To confirm a diagnosis, specific tests are conducted. The most common one is erythrocyte sedimentation rate (ESR):
    • It is performed to measure the degree of inflammation in an individual
    • This can be measured by calculating how quickly the blood cells sediment in a laboratory test
    • When an individual has GCA, their blood cells precipitate out at a faster rate. The rate is typically greater than 50mm/hour
  • Another test that can be done to confirm the diagnosis is a temporal artery biopsy. In this test, a small sample of temporal artery tissue is removed by a surgeon and sent to a pathologist, for analysis. The pathologist will study the tissue sample under a microscope, in order to make a diagnosis

In order to make a definitive diagnosis of Giant Cell Arteritis, 3 of the 5 listed criteria must be present:

  • Age above 50 years
  • New-onset headaches: Individuals start developing headaches; such individuals usually do not have a prominent headache in the past
  • Temporal artery tenderness or decreased temporal artery pulse
  • ESR of 50mm/hour, or higher
  • Inflammatory infiltrate in temporal artery biopsy - this finding is reported by a pathologist

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Giant Cell Arteritis?

Complications of Giant Cell Arteritis could include:

  • The most serious complication that can arise due to Giant Cell Arteritis is blindness. This occurs due to blockage of the short posterior ciliary arteries or central retinal artery, caused by inflammation. This inflammation causes blood vessels to narrow, reducing the amount of blood, oxygen, and nutrients, that travel to the tissues surrounding the eyes
  • In some cases, an aortic aneurysm may occur. Aneurysms occur, when a bulge forms in a blood vessel (like the aorta), which decreases the blood flow. When aneurysms grow too large, they can burst and cause internal bleeding, which are life-threatening (ruptured aortic aneurysm)
  • Individuals with GCA may also suffer from a stroke: If a blood clot forms in the artery leading to the brain, it may obstruct the blood flow completely. This can deprive the brain of blood, oxygen, and nutrients, causing a stroke
  • Treatment with corticosteroids can cause various side effects, such as mood swings, weight gain, weakening of bones (osteoporosis), etc.

How is Giant Cell Arteritis Treated?

The treatment measures of Giant Cell Arteritis include:

  • The only known treatment is with corticosteroids. It is essential that the treatment be started immediately in order to prevent permanent tissue damage
  • Typically, these medications are prescribed for over a year, in order to adequately control inflammation treatment. The effect of these medications, however, is almost immediate; there is a feeling of betterment, within days of starting treatment
  • Most corticosteroid medications are initially prescribed, at a high dose. As symptoms improve, the dose is steadily lowered, until there is no longer a need to take any medications
  • Corticosteroids function by reducing the number of inflammatory cells in the body. This means that the immune system will not have as many cells (as before) to attack the body with, thus alleviating the symptoms
  • The use of corticosteroids in treating Giant Cell Arteritis may be problematic, because they cause many unwanted side effects, which may include:
    • Bone weakening and loss, eventually leading to osteoporosis
    • High blood pressure
    • Muscle weakness
    • Weight gain
    • Increased blood sugar levels
    • Weakened immune system leading to infections

How can Giant Cell Arteritis be Prevented?

  • Currently, there are no known preventative measures against Giant Cell Arteritis
  • This is largely due to the fact that some of the foremost predisposing influences, such as an advancing age, female sex, and Caucasian ethnicity, are quite unavoidable

What is the Prognosis of Giant Cell Arteritis? (Outcomes/Resolutions)

  • If Giant Cell Arteritis is promptly noted and diagnosed, the treatment can be started immediately. This is important in ensuring a favorable prognosis
  • In most cases, corticosteroid treatment can be stopped after 1-2 years. By this time, GCA has mostly resolved and will not likely return
  • Without proper diagnosis and treatment, Giant Cell Arteritis autoimmune disorder can even be fatal

Additional and Relevant Useful Information for Giant Cell Arteritis:

The following article link will help you understand a brain stroke.

https://www.dovemed.com/diseases-conditions/brain-stroke/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 24, 2014
Last updated: May 28, 2018