Giant Cell Arteritis

Giant cell arteritis (GCA), also known as temporal arteritis, is a type of vasculitis or arteritis, a group of diseases whose typical feature is inflammation of blood vessels. In the case of GCA, the vessels most commonly involved are the arteries of the scalp and head, especially the arteries over the temples.

Causes and Risk Factors

GCA may overlap with another disease, polymyalgia rheumatica (PMR). 5 to 10% of patients with PMR will at some point be diagnosed with GCA. Looked at the other way, about one-half of patients with GCA will have symptoms of PMR. The two conditions can occur together or separately, with one following the other. GCA affects the same patients as PMR – only older adults (usually over 60), females more than males, and whites much more than nonwhites. Like PMR, the cause of GCA is unknown.

Symptoms

Symptoms of giant cell arteritis may include:

  • New headache, usually in the area of the temples, though headache due to GCA can occur anywhere, including the front, top, and back of the skull.
  • Unusual fatigue
  • Loss of appetite
  • Weight loss
  • A sense of having the flu
  • Fevers
    Sometimes the only indication of GCA is an unexplained fever.
  • Pain in the jaw with chewing, facial and dental pain, or a new cough
  • If GCA involves the blood supply to the eye, vision can be affected.
    Visual symptoms include temporary blurring of eyesight in one eye, double vision, and actual blindness. Loss of vision in GCA can occur suddenly, and usually cannot be reversed. What is very important, however, is that this complication can be prevented with prompt diagnosis and treatment – in fact, if vision is intact at the time treatment for GCA is started, the risk of visual loss is 1% or less.
  • "Large artery" involvement
    Recently it has bee recognized that GCA can also affect "large arteries", such as the aorta (the main artery from the heart) and the subclavian arteries (the main arterial supply to the arms). Involvement of the aorta can eventually leave to a thoracic aortic aneurysm, a ballooning outward of the aorta, rupture of which can be life-threatening. Risk factors for developing aortic aneurysms in GCA are still being sorted out, but it seems the actual occurrence of aneurysms that cause symptoms in patients with prior GCA is low.

Diagnosis

There is no specific blood test or other noninvasive way to confirm the diagnosis of GCA. The sedimentation rate (“sed rate”) and C reactive protein (CRP) – tests for inflammation – ordinarily are significantly elevated, but because other diseases can cause high sedimentation rates, these findings cannot be relied on for proof of the diagnosis of GCA.

To prove the diagnosis of GCA, it has traditionally been necessary to take a small piece of the temporal artery by biopsy and then examine the wall of the artery under the microscope for evidence of inflammation. The temporal artery biopsy is an outpatient procedure, done under local anesthesia, and leaves only a small scar at the hairline in front of the ear that generally cannot be seen.

At Brigham and Women's Hospital, a new technique for diagnosing GCA is available, and involves the use of ultrasound. The advantage of this particular technique is that it is noninvasive, allows for examination of multiple arteries, and provides immediate results. Over the past four years, close to 600 specialized ultrasound examinations for the diagnosis of GCA have been performed.

In the case of "large artery" involvement by GCA, ultrasound and temporal artery biopsy can be negative, in which case PET CT is needed for diagnosis.

Treatment

Treatment for GCA must begin as soon as possible. If the diagnosis is strongly suspected, treatment will be started before the diagnosis can be proved or disproved. Unlike the treatment for PMR, which requires only low dose steroids (or “glucocorticoids”), higher doses of steroids are needed for the treatment of GCA, typically 40-60 mg of Prednisone per day. Headaches and other symptoms subside quickly, and the sedimentation rate and C reactive protein decline to normal range.

The high dose of steroids is usually kept up for 2 weeks and then slowly tapered. The speed of the taper will be adjusted if there are recurring symptoms or significant rises in the sed rate or CRP, but in most cases the Prednisone dose can be reduced to about 5-10 mg per day over several months, and can eventually be discontinued entirely. Later recurrences of GCA are rare.

If there is no loss of vision when the diagnosis of GCA is made, and if treatment is promptly started with daily, high-dose Prednisone, the risk of subsequent visual loss is essentially abolished.

Higher doses of steroids side-effects are associated more with side-effects, which is attentively monitored by the physician. To protect against bone loss, supplements of calcium and vitamin D are recommended, and are often supplemented with bisphosphonate medications (alendronate, Reclast, and others). Some of the side effects from high dose steroids – for example, jitteriness and weight gain – can be unpleasant, but are reversible, and subside as the steroid doses are reduced to lower levels.

A newer advance in the treatment of GCA is tocilizumab (Actemra), which is administered either by self-injection or by intravenous infusion with Prednisone. Tocilizumab can be used if side-effects from Prednisone develop or if there are recurring symptoms during the course of the Prednisone taper; it can also be started in certain situations at the same time as Prednisone if side effects are anticipated, such as pre-existing diabetes mellitus or significant osteoporosis. Tocilizumab is a biologic agent, and can have its own side-effects, including an increased risk of infection.

Contact Us

The diagnosis of GCA is a matter of urgency, because of the risk of sight loss. Expedited evaluation for the diagnosis of GCA, which includes consultation by a rheumatologist and an ultrasound of the temporal alterities, can be obtained through the Brigham and Women’s Fast Track Clinic for the Diagnosis of Giant Cell Arteritis.

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