Gene G Hunder, MD
John H Stone, MD, MPH
Paul L Romain, MD
INTRODUCTION — Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatory conditions that affect different parts of the body. They are frequently discussed together because:
- Polymyalgia rheumatica occurs in about 50 percent of people with giant cell arteritis
- Approximately 15 to 30 percent of people with polymyalgia rheumatica have giant cell arteritis
Some people with one disorder later develop symptoms of the other.
More detailed information about polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) is available by subscription. (See “Clinical manifestations and diagnosis of polymyalgia rheumatica” and “Diagnosis of giant cell (temporal) arteritis”.)
POLYMYALGIA RHEUMATICA — The most common symptoms of polymyalgia rheumatica (PMR) are:
- Aching and morning stiffness in the shoulder, hips, neck, and upper body. Some people have trouble getting dressed (eg, fastening a bra, putting on a shirt or jacket, or pulling on stockings).
- Swelling or stiffness in the larger joints, such as the knees, or small joints of the hands and feet.
- Some people develop swelling in the hands, wrists, ankles, or the tops of the feet. Swelling in the wrist can lead to numbness, tingling, and pain in the hands (called carpal tunnel syndrome).
- Feeling of tiredness, poor appetite, weight loss, or fever.
Polymyalgia rheumatica diagnosis — A combination of approaches are used to diagnose polymyalgia rheumatica, including an exam, lab tests, and imaging tests (X-ray, MRI, etc). (See “Clinical manifestations and diagnosis of polymyalgia rheumatica”.)
Most people who are diagnosed with PMR:
- Are age 50 years or older when symptoms begin.
- Have aching and morning stiffness (lasting 30 minutes or more) on both sides of the body, persisting for at least one month and involving at least two of the following three areas: neck or upper body, shoulders or upper arms, and hips or upper thighs.
- Have an elevated level of inflammation, which can be measured with a blood test (such as the erythrocyte sedimentation rate).
Polymyalgia rheumatica treatment — Polymyalgia rheumatica responds quickly to medicines that reduce inflammation, such as glucocorticoids (also called steroids). Prednisone and prednisolone are two commonly used glucocorticoids.
During your treatment, it is very important to call your doctor or nurse if you develop any new symptoms that could be caused by giant cell arteritis, such as a change in vision, headaches, jaw or arm pain or weakness, or a new cough. (See ‘Giant cell arteritis symptoms’ below.)
Glucocorticoids — Most people feel better after the first few doses of a glucocorticoid. Your doctor or nurse will gradually decrease the dose every two to four weeks to find the minimum dose that is needed to prevent symptoms. Most people need to take glucocorticoids for one to two years, although some people require an even longer course of treatment.
Glucocorticoids can have serious side effects when taken for long periods of time. Side effects can include weight gain, weakening of the bones, and diabetes. Taking the lowest possible dose for the shortest possible time can help to lower the chance of these side effects.
As the dose of the glucocorticoid is reduced, it is common to develop pain and swelling again. Decreasing the dose slowly can minimize these symptoms.
GIANT CELL ARTERITIS — Giant cell arteritis, also called GCA or temporal arteritis, is a type of blood vessel inflammation that damages medium- and large-sized arteries. The most commonly affected arteries are those that start in the neck and travel into the head.
How are polymyalgia rheumatica and giant cell arteritis related? — About one-half of people with giant cell arteritis have polymyalgia rheumatica. About 15 to 30 percent of people with polymyalgia rheumatic have giant cell arteritis. It is not clear why the conditions often occur together.
Giant cell arteritis symptoms — Symptoms of giant cell arteritis tend to come on slowly. The most common symptoms include headaches, jaw or arm pain or weakness, difficulty seeing clearly, or a new cough. (See “Clinical manifestations of giant cell (temporal) arteritis”.)
Other symptoms can include fever (usually less than 101ºF or 38ºC), feeling tired, and losing weight.
- Headache — The pain, which may be mild or severe, tends to be located over the temples but may be in the front or back of the skull (figure 1). Some people also notice tenderness in the scalp.
- Jaw pain or weakness (claudication) — Nearly one-half of people with giant cell arteritis suffer from jaw pain or weakness. This is also called claudication. Claudication develops because of decreased blood flow to the muscles involved in chewing.
- Vision loss — About 20 percent of people with giant cell arteritis develop partial or complete blindness. Changes in vision may be a first sign of GCA. Vision loss usually begins suddenly with difficulty seeing out of one eye. If untreated, vision in the second eye can also be affected. It is rare, however, to become completely blind in both eyes.
- Arm claudication — Arm claudication causes pain in the arm, especially when moving the arm. It can develop when the arteries to the arm are narrowed.
- Upper respiratory complaints — About 10 percent of people with giant cell arteritis develop a dry cough or a sore throat.
- Thoracic aortic aneurysms — A thoracic aortic aneurysm occurs when the main artery that carries blood from the heart, the aorta, develops a weak spot and balloons outward (figure 2). This does not cause symptoms. However, aneurysms can be life threatening if they burst.
People with giant cell arteritis are usually tested for this type of aneurysm with a once-yearly chest X-ray. (See “Management and outcome of thoracic aortic aneurysm”.)
Giant cell arteritis diagnosis — Your doctor or nurse might perform tests for GCA if you have some of the symptoms listed above. He or she will perform an exam and order some blood tests. If the blood tests show signs of GCA, you will need a biopsy of one or both arteries in the temples (figure 1) and may need imaging tests (eg, an x-ray, ultrasound, or MRI).
Giant cell arteritis treatment
Glucocorticoid — Glucocorticoid (also called steroid) treatment is usually recommended once the diagnosis of giant cell arteritis is strongly suspected, even if it has not yet been proven by biopsy.
Glucocorticoid treatment may be continued, even if artery biopsies performed later reveal no evidence of arteritis. This is especially true if your doctor strongly suspects that you have giant cell arteritis. If there is no evidence of arteritis on biopsy, and your doctor is not convinced you have giant cell arteritis, he or she might recommend decreasing the dose of glucocorticoids or stopping them completely. (See “Treatment of giant cell (temporal) arteritis”.)
Many people continue taking glucocorticoids for one to two years, and occasionally for even longer. The dose of glucocorticoids can begin to be tapered once the disease begins to go into remission. This is done slowly and carefully so that symptoms of giant cell arteritis do not recur.
Methotrexate — Methotrexate is a medicine that was originally designed to treat cancer. When used in much lower doses, methotrexate works to reduce inflammation. It is sometimes used along with glucocorticoids to treat giant cell arteritis.
Antiplatelet treatment — Aspirin is a medication sometimes recommended for people with giant cell arteritis who have an increased risk of blood clots. Aspirin inhibits the clumping of platelets; platelets are tiny cell fragments in the blood that have a role in blood clotting. Under normal circumstances, platelets clump together and help form blood clots to stop bleeding when needed.
There is some evidence that people with giant cell arteritis who take low-dose aspirin have a lower risk of vision loss related to blockage of blood flow in blood vessels of the eye. Based upon these observations and the relatively small risk of low-dose aspirin, most experts recommend that everyone with giant cell arteritis take low-dose aspirin.
Polymyalgia rheumatica — In most people, polymyalgia rheumatica tends to run its course over months to years, and glucocorticoid therapy can eventually be stopped.
Giant cell arteritis — Giant cell arteritis also tends to run its course over several months to several years. However, giant cell arteritis can reappear for no reason during the first year or two after it is diagnosed. The dose of glucocorticoids can eventually be reduced and then stopped in most patients. A small group of people with giant cell arteritis have more chronic disease and require a low dose of glucocorticoids for a number of years.
WHERE TO GET MORE INFORMATION — Your healthcare provider is the best source of information for questions and concerns related to your medical problem.
This article will be updated as needed every four months on our Web site (www.uptodate.com/patients).
Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.
Professional Level Information:
Clinical manifestations and diagnosis of polymyalgia rheumatica
Clinical manifestations of giant cell (temporal) arteritis
Diagnosis of giant cell (temporal) arteritis
Treatment of giant cell (temporal) arteritis
Treatment of polymyalgia rheumatica
Management and outcome of thoracic aortic aneurysm
The following organizations also provide reliable health information.
- National Library of Medicine
- National Institute of Arthritis and Musculoskeletal and Skin Disease
- American College of Rheumatology
- Rodriguez-Valverde V, Sarabia JM, González-Gay MA, et al. Risk factors and predictive models of giant cell arteritis in polymyalgia rheumatica. Am J Med 1997; 102:331.
- Salvarani C, Cantini F, Boiardi L, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 2002; 347:261.
- Brooks RC, McGee SR. Diagnostic dilemmas in polymyalgia rheumatica. Arch Intern Med 1997; 157:162.
- Foroozan R, Deramo VA, Buono LM, et al. Recovery of visual function in patients with biopsy-proven giant cell arteritis. Ophthalmology 2003; 110:539.
- Matteson EL, Gold KN, Bloch DA, Hunder GG. Long-term survival of patients with giant cell arteritis in the American College of Rheumatology giant cell arteritis classification criteria cohort. Am J Med 1996; 100:193.