Temporal Arteritis

Definition

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 to major symptoms, 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.

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.

Vasculitis— An inflammation of the blood vessels.

Resources

ORGANIZATIONS

National Headache Foundation. 428 W. St. James Place, Chicago, IL 60614. (800) 843-2256. 〈http://www.headaches.org〉.

Temporal arteritis

Definition

Temporal arteritis is a disease that causes inflammation and sometimes blockage of medium and large arteries in the head (often near the side of the head or temples).

Description

The mechanism responsible for temporal arteritis (also called giant cell arteritis) is complex and can affect medium and large size arteries, but commonly strikes the temporal artery causing temporal located headaches . In affected arteries there is an abnormal reaction that causes the infiltration of immune cells, such as lymphocytes, multinucleated giant cells, and plasma cells. Frequently the arteries in the head and neck are involved, but vasopathy can extend to the carotids and aorta. The abnormal mechanism is a cell-mediated immune response that is abnormally directed on an antigen (a foreign protein) near the elastic tissue component of an arterial wall. This immune response causes an infiltration of immune cells in an artery which could damage or even completely block the affected blood vessel. The exact cause of temporal arteritis (TA) is unknown. TA can be serious in cases where there is involvement of blood vessels that supply blood to the affected eye (i.e., posterior ciliary artery a branch of the ophthalmic artery) which can cause visual impairment.

Demographics

The disorder is more commonly observed in persons older than 50 years. TA occurs frequently with the occurrence ranging from 10 in 100,000 to 50 in 100,000. Internationally, there seems to be a higher incidence in countries higher in northern climates. The disorder occurs more frequently in Caucasian persons of northern European descent. TA rarely occurs in Blacks and Asians and it is four to six times more frequent in women than men. The mean age of onset is 70 years and the disorder is rarely seen in persons younger than 50 years. Long term survival is the same as for the general population. Visual loss is the most worrisome complication and can occur in over 50% of persons who are untreated, which could result in blindness for 20–50% of these patients.

Causes and symptoms

The cause of TA is not known. It is thought to be due to an immune cell response that attacks a foreign chemical (called an antigen) in the elastic layer of arteries in the head and neck.

In over 85% of affected persons, the most universal symptom is headache. The headache is severe and tends to be on one side (unilateral), and worsens at night. The pain tends to increase as days go by. Visual impairment may be the first presenting symptom since approximately 50% of patients complain of a sudden and painless visual loss. Loss of vision may be transient or permanent and blindness can occur if the condition is untreated.

In approximately 65% of persons, jaw claudication is prominent when chewing, swallowing or talking. Patients may have low grade fever and the effected arteries may be tender, warm, pulseless, dilated and thickened. Other symptoms that can occur include cough, anorexia, muscle aches, malaise, difficulty hearing, fatigue , fever/sweats and depression .

Diagnosis

Criteria for the diagnosis of TA were established in 1990 by the American College of Rheumatology. A diagnosis based on criteria for giant cell arteritis includes the presence of three of the following five items:

• new onset of headache or localized pain in the head region
• temporal artery tenderness to palpation
• development of symptoms in a person over 50 years of age
• lab result of over 50 for a special test called the Westergren Erythrocyte Sedimentation rate (WESR)
• decreased pulsations in head arteries, which cannot be attributed to arteriosclerotic disease of neck (cervical) arteries

A definitive diagnosis is made by the temporal artery biopsy which can be performed as an outpatient procedure (same day surgery).

Blood tests may reveal a high white blood cell count (leukocytosis), mild anemia or an increase in platelet cells (thrombocytosis), which are responsible for blood coagulation. Approximately 50% of patients affected with temporal arteritis have abnormal liver function tests. Chest radiograph may be useful to detect involvement of a chest (thoracic) artery. Ocular pneumoplethysmography can help make the diagnosis of temporal arteritis. Multiple biopsies may be indicated if initial findings are negative, but the suspicion for this diagnosis remains high.

Treatment team

The condition can be diagnosed by a primary care provider. Consultations may be indicated with an ophthalmologist (if there are visual complications). Generally an internist or rheumatologist directs the general care for systemic symptoms.

Treatment

Oral steroids are effective treatment for TA. Treatment is critical and important to avoid vision loss. Treatment should be initiated based on clinical suspicion and should not be delayed for biopsy results. The use of steroid or prednisone can be initially given at a dose of 60 to 100 mg per day. The dose can be tapered down in an individualized manner at a rate of approximately 10% per week, while concurrently taking into account symptomatic state and lab result improvement.

Recovery and rehabilitation

Most patients can be treated on an outpatient basis, with steroids, and symptoms usually begin to resolve within one to three days. Patients may require oral steroid medication for up to one year, depending on individual response. Nonsteroidal anti-inflammatory drugs may provide some pain relief.

Clinical trials

A clinical trial sponsored by the Cleveland Clinic Foundation Hospital and the National Institute of Health concerning the treatment of TA. The study is a multi-institutional project which includes medical centers within the United States and in several countries overseas. Full details can be obtained from the website: <http://www.clinicaltrials.gov>.

Prognosis

The condition is self-limiting and can last up to two years. Treatment with corticosteroids produces relief of symptoms and can help with visual impairment.

Special concerns

A diagnosis of TA can be missed. The disorder should be suspected in older patients with a high erythrocyte sedimentation rate (ESR), even if other evidence is absent.

Resources

BOOKS

Goetz, Christopher G., et al., eds. Textbook of Clinical Neurology. 1st ed. Philadelphia: W. B. Saunders Company, 1999.

Goldman, Lee, et al. Cecil's Textbook of Medicine. 21st ed. Philadelphia: W. B. Saunders Company, 2000.

Noble, John., et al., eds. Textbook of Primary Care Medicine. 3rd ed. St. Louis: Mosby, Inc., 2001.

WEBSITES

American Heart Association. <http://www.americanheart.org>.

ORGANIZATIONS

National Heart, Lung, and Blood Institute, Building 31, Room 5A52. 31 Center Drive MSC 2486, Bethesda, MD 20892. (301) 592-8573; Fax: (301) 592-8563. [email protected]. <http://www.nhlbi.nih.gov>.

Laith Farid Gulli, MD

Robert Ramirez, DO

Alfredo Mori, MBBS