Section 5. Bone, Joint, and Muscle Disorders

Chapter 69. Vasculitic Disorders of Connective Tissue

Topics: Introduction | Polyarteritis Nodosa | Temporal Arteritis | Polymyalgia Rheumatica | Wegener's Granulomatosis | Behçet's Syndrome

Temporal Arteritis

Temporal (giant cell) arteritis is chronic inflammation of large arteries, typically of the temporal artery, an artery on the side of the scalp.

This disorder affects about 1 of 1,000 people older than 50 and slightly more women than men. Its cause is unknown. Temporal arteritis often occurs with polymyalgia rheumatica.

Symptoms

The symptoms vary, depending on which arteries are affected. Typically, the large arteries to the head are affected, and a severe headache usually develops suddenly at the temples or back of the head. The blood vessels in the temple may feel swollen and bumpy. The scalp may feel painful when the hair is brushed. Double vision, blurred vision, large blind spots, blindness of one eye, or other eye problems may develop. The greatest danger is permanent blindness, which can occur suddenly if the blood supply to the optic nerve is blocked.

Characteristically, the jaw, chewing muscles, and tongue may hurt when eating or speaking. Other symptoms may include severe pain in the neck, shoulders, and hip, which also occurs in polymyalgia rheumatica.

Diagnosis and Treatment

Doctors base their diagnosis on the symptoms and a physical examination and confirm it by performing a biopsy of the temporal artery, located in the temple. Blood tests are also helpful, usually detecting anemia and a very high erythrocyte sedimentation rate (ESR), which indicates inflammation.

See the figure Biopsy of the Temporal Artery.

Because temporal arteritis causes blindness in 20% of untreated people, treatment must begin as soon as the disease is suspected. Treatment is usually started even before a biopsy is performed; treatment does not affect the biopsy results as long as the biopsy is performed within several days of starting treatment. Prednisone, a corticosteroid, is highly effective. Initially, the dose is high--to stop the inflammation in the blood vessels; after several weeks, doctors slowly taper the dose if the person is improving. Some people can stop taking prednisone within a few years, but many need very low doses for many years to control symptoms and prevent blindness.