|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1986, 1988, 1994, 1998, 2001, 2002, 2007
Giant cell arteritis is a chronic inflammatory disease characterized by the progressive inflammation of many arteries of the body (panarteritis). Granular material and abnormally large cells (giant cells) accumulate in the elastic lining of the arteries. Chronic inflammation is sometimes confined to the different branches of the heart's main artery (aorta) and any large arteries can become inflamed. However, the temporal arteries of the head are most frequently affected (temporal arteritis). In rare cases, veins may also be affected by giant cell arteritis.
The symptoms of giant cell arteritis may include stiffness, muscle pain, fever, and/or headaches. The exact cause of this disease is not fully understood, although it is thought to be an autoimmune disease that occurs when the body's, own immune system attacks healthy tissue.
Giant cell arteritis is closely related to polymyalgia rheumatica, another inflammatory disorder. These two disorders have been described in the medical literature as possible variants of the same disease process. Some researchers believe they represent different ends of a disease continuum. The exact nature of the association is not fully understood.
The most common symptoms of giant cell arteritis are headaches and tenderness of the temples due to inflammation of the arteries on either side of the head (temporal arteries). This characteristic finding is why giant cell arteritis is sometimes called temporal arteritis.
Severe throbbing and stabbing pain on both sides of the head (temporal area) are frequently accompanied by redness, swelling, and/or tenderness on the scalp. Some affected individuals may feel rhythmic pulsations in the temporal arteries. The scalp may be tender in these reddened areas, and the temporal arteries may appear twisted and knotted under the scalp. Some people may also have trouble chewing. Other symptoms relate to difficulties with the eyes and visual impairment (retinal vessel disease). About 50 percent of people may experience a variety of visual symptoms, but only 10 percent of these people experience varying degrees of visual loss or blindness.
Additional symptoms of giant cell arteritis may begin gradually or suddenly and may resemble those of the flu. Symptoms may include low-grade fever, muscle pain (myalgia), low levels of circulating red blood cells (mild anemia), and/or severe stiffness, especially in the morning or after a period of inactivity (gel phenomenon). A few people with this disorder may experience respiratory symptoms including cough, sore throat, and/or hoarseness. These symptoms are often confused with other more common respiratory infections, making the diagnosis of giant cell arteritis difficult in some people.
Giant cell arteritis may be localized or widespread. When giant cell arteritis affects other medium or large arteries of the body (i.e., coronary, carotid, subclavian, pulmonary, renal, and mesenteric arteries), serious complications may develop. These may include blindness, stroke (cerebral vascular accident), blockage of the artery that carries blood from the heart (coronary artery occlusion), and/or the lack of fresh blood supply to the arms and legs (arterial insufficiency).
In approximately 50 percent of cases, individuals with giant cell arteritis develop symptoms related to polymyalgia rheumatica. Polymyaglia rheumatica is a rare form of arteritis, may affect the lower back, thighs, neck, shoulders, and/or hips. Symptoms may include stiffness that is most severe in the morning. Joint inflammation (synovitis), especially in the knees, is also common. There is some disagreement in the medical literature as to whether polymyalgia rheumatica and giant cell arteritis are actually part of a disease spectrum or whether they are related, but distinct disorders. (For more information on this disorder, choose "polymyalgia rheumatica" as your search term in the Rare Disease Database.)
The exact cause of giant cell arteritis is not known. The immune system has been implicated in some studies, but a direct relationship has not been positively established. Autoimmune disorders are caused when the body's natural defenses against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.
Familial cases of giant cell arteritis have also been reported. Medical research indicates that some people with giant cell arteritis may have a genetic predisposition to the disease. A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease.
In giant cell arteritis, affected blood vessels become inflamed and swell. The cause for this reaction is not known. Inflammation of blood vessels causes the symptoms of giant cell arteritis such as headaches because of inflammation of the temporal arteries of the head. Swelling also causes narrowing of the blood vessels, which, in turn, results in diminished blood flow.
Giant cell arteritis is an inflammatory disease that affects twice as many females as males. It occurs in approximately 24 in 100,000 people over the age of 50 years. Giant cell arteritis is estimated to affect 110,000 individuals in the United States. The disease usually develops between the ages of 50 to 90 years. The average age of onset is about 70 years. Americans of European descent are more frequently affected than those of African or other ethnic heritage.
Symptoms of the following disorders can be similar to those of giant cell arteritis. Comparisons may be useful for a differential diagnosis:
Polymyalgia rheumatica is a rare chronic inflammatory disease characterized by muscle pain, stiffness, fatigue, and/or fever. Onset is usually abrupt, with pain and stiffness in the neck, shoulders, upper arms, lower back, hips, and/or thighs. Stiffness and pain usually affect both sides of the body and are most severe in the morning or after periods of rest or inactivity (gel phenomenon). Other symptoms may include loss of appetite, weight loss, a general feeling of ill health (malaise), and/or depression. The exact cause of polymyalgia rheumatica is not known. (For more information on this disorder, choose "polymyalgia rheumatica" as your search term in the Rare Disease Database.)
Polymyositis is a rare disease characterized by inflammation and degenerative changes in the muscle fibers and the supporting connective tissue. Muscle weakness may occur rapidly and affect the neck, trunk, and upper arms and legs. Joint pain, swelling, and tenderness may be present. Eventually it becomes difficult for affected individuals to rise from a sitting position. Climbing stairs, lifting objects, and reaching overhead may also be uncomfortable. The exact cause of polymyositis is not known, although it may be due to the interaction of inherited, viral, and environmental factors. (For more information on this disorder, choose "polymyositis" as your search term in the Rare Disease Database.)
Takayasu arteritis is a rare disorder characterized by inflammation of the large elastic arteries of the vascular system. The main arteries of the heart (aorta) and lungs (pulmonary) are most often affected. Symptoms may be similar to those of an acute infectious illness and may include fever, profound loss of appetite, a general feeling of ill health (malaise), muscle pain (myalgias), and joint pain (arthralgias). When the aorta and the major arteries in the neck (carotids) are affected, symptoms may include lightheadedness, dizziness, and brief moments of unconsciousness (syncope). The exact cause of takayasu arteritis is not known. (For more information on this disorder, choose "takayasu arteritis" as your search term in the Rare Disease Database.)
Vasculitis is a general term used to describe a common condition of inflammation in the walls of blood vessels, including arteries, veins, and capillaries. This inflammation results in a narrowing of the vessels (stenosis) and can obstruct the flow of blood to various organs of the body (ischemia). The symptoms of vasculitis vary greatly, depending on the location and severity of the inflammation. Symptoms may include muscle pain, joint pain, fever, weight loss, headache, night sweats, and/or generalized weakness. Some affected individuals may develop hoarseness and sores in their mouths. When vasculitis affects the respiratory system, symptoms may include shortness of breath, nosebleeds, a runny nose, and/or coughing. (For more information on this disorder, choose "vasculitis" as your search term in the Rare Disease Database.)
Mixed connective tissue disease (MCTD) is a rare inflammatory disorder of the connective tissue. The symptoms of this disorder overlap with those of giant cell arteritis, lupus (systemic lupus erythematosus), scleroderma, and polymyositis. Early symptoms may include a fever of unknown origin, painfully cold fingers in response to cold (raynaud phenomenon), swollen hands, fatigue, and/or nondeforming arthritis. Arthritis occurs in almost every case of MCTD, but rarely results in deformities similar to those seen in rheumatoid arthritis. Muscle pain and skin rashes are also very common. (For more information on this disorder, choose "mixed connective tissue disease" as your search term in the Rare Disease Database.)
The diagnosis of giant cell arteritis is confirmed by a complete clinical evaluation, angiography, arterial biopsy, and blood tests. X-ray studies of the affected areas of the scalp are performed with a special contrast dye (angiography). These studies may show unusual areas of obstruction or narrowing (stenosis) in the temporal arteries. In some cases, a biopsy may be performed. Tissue samples are usually taken from the area of the artery that appears most twisted or knotted. Microscopic examination of tissue samples usually reveals the presence of abnormal giant cells, granular material (granulomatous inflammation), and tissue loss (focal necrosis). A specialized blood test, erythrocyte sedimentation rate (ESR), is usually elevated in people with giant cell arteritis.
The treatment of giant cell arteritis should start when the diagnosis is suspected. Prompt treatment may help to avoid serious complications such as blindness and coronary blockage (occlusion). High doses of corticosteroids (i.e., prednisone at 60 to 80 mg/day) may control initial local and systemic symptoms. This therapy is continued until symptoms subside and blood tests (e.g., erythrocyte sedimentation rate) have returned to normal, which generally occurs in about 2 to 4 weeks. Corticosteroid therapy is usually continued at gradually reduced doses for about 2 years or longer. This low dose therapy helps to reduce the risk of recurrence of giant cell arteritis.
The activity of giant cell arteritis can then be monitored by periodic blood testing of the sedimentation rate (ESR). A rise in the sedimentation rate along with the return of symptoms, especially headaches, usually signals a recurrence of the disease. Other treatment is symptomatic and supportive.
Some people with giant cell arteritis may not be able to tolerate high-dose corticosteroid drug therapy. For these individuals, other drugs such as azathioprine, methotrexate, and dapsone have been studied as possible treatment alternatives. More study is needed to determine the long-term safety and effectiveness of these drugs for the treatment of giant cell arteritis.
The drug infliximab is being studied as a potential treatment for individuals with giant cell arteritis. Further clinical study is necessary to determine the long-term safety and effectiveness of this potential treatment for individuals with giant cell arteritis.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors. If you're interested in becoming a member, please contact Susan Olivo, Membership Manager, at firstname.lastname@example.org.)
Wyngaarden JB and Smith LH, eds. Cecil Textbook of Medicine, 20th ed. Philadelphia, PA:W.B. Saunders Company; 1996:1498-1500.
Beers MH and Berkow R, eds. The Merck Manual. 17th ed. Whitehouse Station, MJ:Merck Research Laboratories. 1999:438-439.
Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:1398,1917.
Magalini S, Magalini S, and de Francisci G, eds. Dictionary of Medical Syndromes. 4th ed. Philadelphia, PA: Lippincott-Raven;1997:386.
Hoffman GS, et al., A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis. Arthritis Rheum. 20002;46:1309-18.
Salvarani C, et al., Medical progress: polymyalgia rheumatica and giant-cell arteritis. N Engl J Med. 2002;347:261-71.
Barilla-LaBarca ML, et al., Polymyalgia rheumatic/temporal arteritis: recent advances. Curr Rheumatol Rep. 2002;4:39-46.
Cantini F, et al., Treatment of longstanding active giant cell arteritis with infliximab: report of four cases. Arthritis Rheum. 2001;44:2933-5.
Jover JA, et al. Combined treatment of giant-cell arteritis with methotrexate and prednisone: A randomized, double-blind, placebo-controlled trial. Ann Intern Med. 2001;134:106-14.
Myers KA et al. Ultrasonography in temporal arteritis. New Eng J. Med. 1998;338:760-61.
Schmidt et al. Color Duplex ultrasonography in the diagnosis of temporal arteritis. New Eng J Med. 1997;337:1336-42.
Wernick et al. Familial giant cell arteritis: report of an hla-typed sibling pair and a review of the literature. Clin Exp Rheumatol. 1994;12:63-66.
Nordboerg E, et al. Giant Cell Arteritis Epidemiology and Treatment. Drugs Aging. 1994;4:135-44.
Kyle V et al. The clinical and laboratory course of polymyalgia rheumatica/giant cell arteritis after the first two months of treatment. Ann Rheum Dis. 1993;52:847-50.
DiBartolomeo AG et al. Giant cell arteritis and polymyalgia rheumatica. Postgrad Med. 1992;91:107-09.
Nordboerg E, et al. Giant Cell Arteritis. Curr Opin Rheumatol.1992;4:23-30.
FROM THE INTERNET
Roque MR. Giant Cell Arteritis. Emedicine Journal, January 10 2006. Available at: http://www.emedicine.com/oph/topic254.htm Accessed on: May 2, 2007.
Mayo Clinic for Medical Education and Research. Giant Cell Arteritis. July 19, 2006. Available at: http://www.mayoclinic.com/health/giant-cell-arteritis/DS00440 Accessed On: May 2, 2007.
Report last updated: 2007/08/07 00:00:00 GMT+0