Giant Cell Arteritis - Know It All!

Editorial

Giant Cell Arteritis — Know It All!

All you need to know about Temporal Arteritis.

Giant Cell Arteritis
Giant Cell Arteritis

Know your ailment well, so you can manage it better!!

Here we come with Giant Cell Arteritis today!

Overview:

Giant Cell Arteritis is also known as Temporal Arteritis.

GCA is a form of vasculitis, or arteritis, a group of diseases whose main symptom is blood vessel inflammation. The vessels most frequently involved in GCA are the scalp and head arteries, particularly the arteries above the temples, which is why another word for GCA is “temporal arteritis.”

Giant Cell Arteritis
Giant Cell Arteritis

GCA can overlap with Rheumatic Polymyalgia (PMR). At some stage, GCA will be diagnosed in 5–15 percent of PMR patients. Approximately 50 percent of GCA patients have PMR symptoms. The two conditions can occur simultaneously or by themselves. Often it affects the same patient groups as PMR. It occurs only in adults, typically over 50 years old, more in women than men, and more in whites than in non-whites.

What happens in Giant Cell Arteritis:

Giant cell arteritis causes narrowed arteries, limiting the flow of blood. The disease mainly affects arteries on either side of the head while other blood vessels can also get inflamed.

Untreated giant cell arteritis can potentially cause serious complications, including permanent loss of vision and stroke. Reporting any symptoms to the doctor and seeking early care to avoid irreversible damage to the tissue is essential.

Symptoms of GCA:

GCA’s most common symptom (what you feel) is a new headache, usually around the eyes, but headache due to GCA can occur anywhere from the skull‘s forehead, top and back. Symptoms such as exhaustion, lack of appetite, weight loss or a flu-like sensation are almost as common. Chewing can cause pain to the jaw. The only symptom of GCA is, often, unexplained fever. Less common signs include eye, tongue or throat pains.

If GCA spreads to the eye’s blood supply, it can impair your eyesight. Vision disorders may involve intermittent blurring, blindness or double vision. Permanent vision loss in GCA may occur unexpectedly but this complication can be avoided by careful care. Indeed, if the vision of a patient is fine when they begin treatment, the chance of eventual sight loss is 1 in 100 or less. It is important that patients who have active or inactive PMR report to their doctors urgently any signs of new headache, changes in vision or jaw pain. As with PMR, it is not known what causes GCA.

Giant Cell Arteritis
Giant Cell Arteritis

Giant cell arteritis’s most common symptoms are head pain and tenderness — often severe — which typically affects both temples. Head pain can gradually escalate, come and go or momentarily subside.

Generally, signs and symptoms of giant cell arteritis include:

  • Persistent, severe head pain, usually in your temple area
  • Scalp tenderness
  • Jaw pain when you chew or open your mouth wide
  • Fever
  • Fatigue
  • Unintended weight loss
  • Vision loss or double vision, particularly in people who also have jaw pain
  • Sudden, permanent loss of vision in one eye

Pain and stiffness in the spine, shoulders, and hips are typical symptoms of a related condition, rheumatic polymyalgia. There is also polymyalgia rheumatic in about 50 percent of people with giant cell arteritis.

Causes:

Giant cell arteritis inflames the lining of arteries, causing them to swell. This swelling narrows your blood vessels, reducing the amount of blood that reaches your body’s tissues — and therefore, oxygen and vital nutrients.

Nearly any large or medium-sized artery can be affected, but the most frequent swelling occurs in temple arteries. These are just right in front of your ears and keep going up into your scalp.

It is not known what causes these arteries to become inflamed but it is thought that the immune system will involve abnormal attacks on artery walls. Some genes and environmental factors could make you more susceptible to the condition.

Complications:

Giant cell arteritis can cause serious complications, including:

  • Blindness. Diminished blood flow to your eyes can cause sudden, painless vision loss in one or, rarely, both eyes. The loss of vision is usually permanent.
  • Aortic aneurysm. An aneurysm is a bulge formed in a damaged blood vessel, usually in a large artery running down your chest and abdominal (aorta) centre. An aortic aneurysm may burst, causing internal bleeding that is life threatening.Since this complication can occur even years after giant cell arteritis has been diagnosed, your doctor can monitor your aorta with annual chest X-rays or other imaging tests, such as ultrasound and CT.
  • Stroke. This is an uncommon complication of giant cell arteritis.

Giant Cell Arteritis
Giant Cell Arteritis

Risk Factors:

Several factors can increase your risk of developing giant cell arteritis, including:

  • Age. Giant cell arteritis affects adults only, and rarely those under 50. Most people with this condition develop signs and symptoms between the ages of 70 and 80.
  • Sex. Women are about two times more likely to develop the condition than men are.
  • Race and geographic region. Giant cell arteritis is most common among white people in Northern European populations or of Scandinavian descent.
  • Polymyalgia rheumatica. Having polymyalgia rheumatica puts you at increased risk of developing giant cell arteritis.
  • Family history. Sometimes the condition runs in families.

Who Develops this disease?

  • Women are more likely than men to develop giant cell arteritis, but men with the disorder are more likely to suffer eye damage that could result in blindness. Giant cell arteritis mostly affects people over the age of 50, with the highest rates at 70 to 80 years of age.

How to diagnose:

Giant cell arteritis can be hard to diagnose, as its early symptoms are similar to those of other common conditions. Your doctor will try to rule out other possible causes of your problem for this reason.

Besides asking about your symptoms and medical history, your doctor will probably do a thorough physical examination, paying particular attention to your temporal arteries. Often one or both of these arteries are tender, with a reduced pulse and a tough, cord-like appearance and feel. Your doctor may recommend certain tests, too.

Blood Tests:

The following tests might be used to help diagnose your condition and to follow your progress during treatment.

  • Erythrocyte sedimentation rate: Commonly known as the sedation rate, this test tests how easily red blood cells fall to the bottom of a blood tube. Red cells that drop quickly can indicate inflammation in your body.
  • C-reactive protein (CRP): This measures a substance which your liver produces when there is inflammation.

Imaging Tests:

These might be used to diagnose giant cell arteritis and to monitor your response to treatment. Tests might include:

  • Doppler ultrasound. This test uses sound waves to produce images of blood flowing through your blood vessels.
  • Magnetic resonance angiography (MRA). This test combines an MRI with the use of a medium of contrast to create clear images of your blood vessels. If you are uncomfortable being enclosed in a small room, let your doctor know in advance because the test is performed in a tube-shaped system.
  • Positron emission tomography (PET). If your doctor suspects that you may be experiencing giant cell arteritis in large arteries such as your aorta, he or she may prescribe PET. This procedure uses an intravenous tracer solution with a small amount of radioactive material in it. A PET scan will provide clear images of your bigger blood vessels and highlight inflammatory areas.

Biopsy:

The best way to confirm a diagnosis of giant cell arteritis is by taking a thin, temporal artery sample (biopsy). This artery is located in front of your face, close to the skin and continues up to your scalp. Using local anaesthesia, the operation is done outpatiently, usually with no pain or scarring. In a laboratory the sample will be studied under a microscope.

If you have giant cell arteritis, the artery often exhibits inflammation that includes abnormally large cells , called giant cells, that give the disease its name. Giant cell arteritis may occur and has a negative biopsy outcome.

If the results are not clear, your doctor may suggest you on the other side of your head for another temporal artery biopsy.

Giant Cell Arteritis
Giant Cell Arteritis

Treatment Options:

GCA therapy should start as soon as possible, due to the possibility of vision loss. If your doctor suspects GCA strongly, care can begin before you get the results of a temporary biopsy of the arteries. Unlike PMR treatment, which needs only low-dose corticosteroids (also known as glucocorticoids), GCA treatment typically includes high corticosteroid dosages. Usually the dosage of prednisone (Deltasone, Orasone, etc.) is 40–60 milligrammes ( mg) per day. Headaches and other symptoms lessen rapidly with treatment, and the rate of sedimentation decreases to a normal range.

The high dose of corticosteroids typically lasts for one month, and then the dose falls steadily. If you have recurrent GCA symptoms or major increases in sedimentation rate, the pace at which your doctor lowers the dose can change. However, in most cases, the dosage of prednisone may be lowered over a few months to around 5–10 mg per day. Patients are typically tapered by one to two years off this drug. Aftercare GCA rarely returns.

In May 2017, GCA was approved for the treatment of tocilizumab (Actemra). This drug can be given every one or two weeks as an intravenous infusion, daily, or as a subcutaneous injection, self-administered by the patient. Tocilizumab has been shown to provide more patients with one year of treatment in remission from GCA, and to require fewer overall prednisone volumes.

Giant Cell Arteritis
Giant Cell Arteritis

Coping with the disease:

Knowing what you can about giant cell arteritis and its treatment will make you feel more at ease with your condition. Your healthcare team will answer your questions, and support groups online may also be of benefit. Know the potential side effects of the drugs you are taking, and report any changes to your doctor’s health.

References:

https://www.niams.nih.gov/health-topics/giant-cell-arteritis

https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Giant-Cell-Arteritis

https://www.mayoclinic.org/diseases-conditions/giant-cell-arteritis/diagnosis-treatment/drc-20372764

By,

Gopala Krishna Varshith,

Content Developer & Editor,

Clipo.