Giant cell (temporal, cranial) arteritis

Alternative names:

cerebral vasculitis

Definition:

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

Causes, incidence, and risk factors:

Cerebral vasculitis (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 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 polymyalgia rheumatica. It has also 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 3 out of 10,000 people. It is more common in women. It is seen most often in those over 50 years old but has been documented in people less than 40. years old. It affects about 1 out of 750 people over 50 years old. It is rare in people of African descent. There is some evidence for a familial pattern of developing the disorder.