A disorder involving inflammation and damage to blood vessels, particularly the large or medium arteries that branch from the external carotid artery of the neck.

Causes, incidence, and risk factors

Giant cell, cranial, or temporal arteritis occurs when there is inflammation and necrosis (death of the tissues) of one or more arteries. It most commonly occurs in the head, especially the temporal arteries that branch from the carotid artery of the neck. However, it can be systemic , affecting multiple medium to large sized arteries anywhere in the body. The cause is unknown but is assumed to be, at least in part, an effect of the immune response . The disorder has been associated with severe infections, high doses of antibiotics, and chronic disorders such as rheumatoid arthritis and systemic lupus erythematosus . The symptoms occur because of inflammation. The disorder may exist independently or may coexist with or follow polymyalgia rheumatica (a disorder characterized by abrupt development of pain and stiffness in the pelvis and shoulder muscles). About 25% of people with giant cell arteritis also experience polymyalgia rheumatica. Giant cell arteritis affects approximately 25 out of 100,000 women and 9 out of 100,000 men. It is seen most often in those over 50 years old, but has been documented in people less than 40. years old. It is rare in people of African descent. There is some evidence for a familial pattern of developing the disorder.

Signs and tests

Palpation

of the head shows scalp sensitivity and often shows a tender, thick, palpable artery on one side of the head. The affected artery may have a weakened

pulse or no pulse . About 40% of people will have other nonspecific symptoms such as respiratory complaints (most frequently dry cough ) or mononeuritis multiplex ( weakness and/or pain of multiple individual nerve groups). Rarely, ocular palsies ( paralysis of eye muscles) may occur. Fever may be the only symptom (the person presents with a persistent fever of unknown origin). Blood tests are nonspecific.

A

sedimentation rate ( ESR ) is almost always very high.

A

hemoglobin or hematocrit may be normal or low.

Liver function tests

may be abnormal if the disorder is

systemic .

Alkaline phosphatase

may be elevated.

A biopsy and analysis of tissue from the affected artery show changes that confirm the diagnosis of cerebral vasculitis in most cases.

Treatment

The goal of treatment is to minimize irreversible tissue damage that may occur because of lack of blood flow (ischemia). Corticosteroids such as Prednisone are commonly prescribed to reduce inflammation. If symptoms suggest cerebral vasculitis, corticosteroid treatment may be started even before a biopsy confirms the diagnosis (to reduce the risk of complications). Aspirin may be recommended in addition to corticosteroids. Medications that suppress the immune system (such as cyclophosphamide) are occasionally prescribed.

Expectations (prognosis)

The disorder usually results in recovery but may require prolonged treatment for 1 to 2 years. Recurrence is possible for several years.

Complications

Common:

side effects of therapy

obstruction of the arteries of the eye, leading to:

blindness

ophthalmoplegia (

weakness or paralysis of one or more eye muscles) Uncommon:

a

stroke

a

TIA

damage to cranial nerves

Calling your health care provider

Call your health care provider if persistent throbbing headache and other symptoms that indicate giant cell arteritis occur.

Prevention

There is no known prevention.