The term temporal arteritis literally means "inflammation of the temporal arteries."As implied by the name, these blood vessels run along the temples after they branch off from the carotid artery in the neck. They provide the blood supply to portions of the scalp, jaw muscles, and salivary glands. Inflammation of these arteries, probably resulting from an abnormal immune reaction, disrupts this blood supply, resulting in a variety of symptoms. They can range from relatively minor—jaw pain or headache—through major—including temporary or permanent blindness.
Temporal arteritis is also called giant cell arteritis or cranial arteritis. It is a rheumatic disease that affects large and medium-sized arteries throughout the body and can occur in a variety of patients. Although the temporal arteries are most commonly affected, other arteries throughout the body may be affected. The disease seems to target arteries containing elastic tissue. Veins are rarely affected. Temporal arteritis is a type of vasculitis.
Description
Temporal arteritis almost always occurs in people over 50, and it becomes more common as people age. About 20 out of 100,000 people over the age of 50 suffer from temporal arteritis. Women are affected twice as often as men. Some authorities say that temporal arteritis is more common in Caucasians (especially Scandinavians) than in people of other races. Close relatives of patients with temporal arteritis may be more likely than others to get the disease.
Patients with temporal arteritis are diagnosed and overlap with a broader disorder called giant cell arteritis. This can affect parts of the body in addition to the scalp, eyes, and jaw. Sometimes the disease can cause restricted circulation to both arms or both legs, producing pain in the affected limbs. With other blood vessels involved, patients with advanced forms of the disease may experience strokes or transient ischemic attacks (TIA). These result in brief episodes of pain caused by decreased blood flow. Even heart attacks are occasionally caused by giant cell arteritis.
Causes and symptoms
This disease is one of a group of diseases in which the linings of large- or medium-sized blood vessels become inflamed. The elastic layer of these vessels is attacked by "giant" cells and chemicals produced by the immune system. This reaction reduces blood flow through the blood vessels, and the limited blood supply causes the symptoms.
The disease usually begins with "flu-like" symptoms, including a mild fever (100–101°F), general body discomfort, and a persistent, dull headache. The scalp may be tender to the touch over the affected blood vessels. Jaw muscles sometimes become painful when the patient chews.
As the disease progresses, more severe symptoms occur. These include blurred vision or temporary blindness that typically lasts ten minutes or less. Eventually, permanent loss of vision can occur. Transient ischemic attacks, strokes, and heart attacks may occur when the disease is far advanced.
Diagnosis
Doctors from a number of specialties develop experience in diagnosing and treating temporal arteritis. These include internists, who treat a broad range of diseases; rheumatologists, who focus on rheumatic diseases; geriatricians, who treat older people; ophthalmologists, who treat eye and vision disorders; neurologists, who treat headaches and problems of the optic nerve; and vascular surgeons, who treat blood vessel problems.
The doctor will generally take a medical history first. The patient can help the doctor tremendously by reviewing all symptoms—both major and minor—from the last two or three months. If possible, the patient should ask family or close friends for help in recalling his/her ailments from recent months. Then the doctor will conduct a complete physical examination. Often, he or she will detect a tender, swollen artery on the scalp.
The doctor will order blood tests as well. A standard and inexpensive test called the erythrocyte sedimentation rate (ESR or "sed" rate) is particularly helpful. Results from this test, which measures inflammation in the body, will almost always be higher than normal. Tests of the red blood cells may show mild anemia. Sometimes blood tests for liver function will also be abnormal.
The definitive diagnostic test is a temporal artery biopsy. A doctor will make one or more tiny incisions under local anesthesia to remove samples of the suspect artery. Under the microscope, a pathologist usually can identify the typical damage caused by temporal arteritis.
Treatment
The mainstay of treatment is a course of corticosteroids (steroid hormones that have an anti-inflammatory effect), usually prednisone. The initial prescription involves a fairly high dose of steroids (40–60 mg/day) which is gradually tapered down to a maintenance dose. Because of the high incidence of blindness in untreated cases, steroid therapy should be started immediately rather than waiting for biopsy results. Patients typically take this maintenance dose for periods of one to three years. Sometimes nonsteroidal anti-inflammatory drugs (NSAIDs) are prescribed for muscle aches or headaches, especially while steroid doses are being reduced.
Prognosis
The outlook for most patients with temporal arteritis is good, especially if the disorder is diagnosed early. Symptoms often diminish within a month once patients begin to take steroids. Although physicians do not talk about a "cure" for temporal arteritis, symptoms typically do not return after a full course of steroid treatment. Unfortunately, if the diagnosis is made late in the disease, lost vision may not return.
Prevention
There is no medically proven approach to prevention. The best way to prevent severe, permanent damage is to obtain expert medical advice if the patient or the family physician suspects this problem.
BOOKS
Diamond, Seymour, and David Dalessio. The Practicing Physician's Guide to Headache. 5th ed. Baltimore: Williams & Wilkins, 1992.
PERIODICALS
Hayreh, S. S., et al. "Giant Cell Arteritis: Validity of Reliability of Various Diagnostic Criteria." American Journal of Ophthalmology 123, no. 3 (1997): 285-296.
Perkins, A. T., and W. Ondo."When to Worry About Headache." Postgraduate Medicine 98, no. 2 (1995): 197-205.
ORGANIZATIONS
National Headache Foundation. 428 W. St. James Place, Chicago, IL 60614. (800) 843-2256. <http://www.headaches.org>.
Richard H. Lampert
KEY TERMS
Anemia—Lower than normal level of red blood cells, or of the oxygen-carrying chemical hemoglobin.
Biopsy—Removal and examination of a sample tissue from the body for diagnostic purposes.
Corticosteroids—A group of hormones, produced naturally by the adrenal gland and other organs. They are used to treat a wide variety of disorders, including many rheumatic disorders.
Erythrocyte sedimentation rate—The speed at which red blood cells sink in a tube of freshly drawn blood, which is a rough measure of clotting disorders or inflammation.
Prednisone—A corticosteroid often used to treat inflammation.
Rheumatic disease—A type of disease involving inflammation of muscles, joints, and other tissues.
Transient ischemic attack—A brief experience of stroke-like symptoms (for instance, numbness, paralysis, problems in speaking or understanding speech) that go away within hours, with no permanent damage. Also known as TIA.
