Patient education: Polymyalgia rheumatica and giant cell (temporal) arteritis (Beyond the Basics)
- William P Docken, MD
William P Docken, MD
- Assistant Professor of Medicine
- Harvard Medical School
Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are linked inflammatory conditions that affect different parts of the body. They are frequently discussed together because:
●PMR occurs in about 50 percent of people with GCA.
●GCA occurs in approximately 10 to 15 percent of people with PMR.
The two disorders do not necessarily occur at the same time.
More detailed information about PMR and GCA is available elsewhere. (See "Clinical manifestations and diagnosis of polymyalgia rheumatica" and "Diagnosis of giant cell (temporal) arteritis".)
The most common symptoms of polymyalgia rheumatica (PMR) are:
●Aching and stiffness in the morning, often for several hours or for a shorter period after rest during the day, located in the shoulders and upper arms, hips and thighs, and back of the neck. Some people have trouble getting dressed (eg, fastening a bra, putting on a shirt or jacket, or pulling on stockings). Difficulty raising the arms above the shoulders is a typical symptom.
●Occasional swelling or stiffness of the small joints of the hands. 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.
Symptoms of PMR usually come on rather abruptly over a period of weeks, sometimes days.
Polymyalgia rheumatica diagnosis — A combination of approaches is used to diagnose PMR, including a history, physical exam, and lab tests. (See "Clinical manifestations and diagnosis of polymyalgia rheumatica".)
Most people who are diagnosed with PMR:
●Are older adults in their 60s or 70s. PMR virtually never occurs under the age of 50.
●Have persisting aching and morning stiffness (lasting 30 minutes or more) on both sides of the body, 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 (the erythrocyte sedimentation rate or C-reactive protein).
Polymyalgia rheumatica treatment — PMR responds quickly to low doses of glucocorticoids (also called steroids), medications that reduce inflammation. Prednisone and prednisolone are two commonly used glucocorticoids. Nonsteroidal antiinflammatory drugs (NSAIDs; such as ibuprofen or naproxen) are ineffective.
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 (GCA), 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 reduce the chance of these side effects.
As the dose of the glucocorticoid is reduced, stiffness and pain can recur. 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 and scalp.
How are polymyalgia rheumatica and giant cell arteritis related? — About one-half of people with GCA have polymyalgia rheumatica (PMR). About 10 to 15 percent of people with PMR have GCA. It is not clear why the conditions often occur together.
Giant cell arteritis symptoms — The most common symptoms include new headaches, jaw or arm pain or weakness, difficulty seeing clearly (especially out of one eye), or a new cough. (See "Clinical manifestations of giant cell (temporal) arteritis".)
Other symptoms can include developing a fever (usually less than 101ºF or 38ºC), feeling tired, and weight loss.
●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 (claudication) – Nearly one-half of people with GCA suffer from jaw pain, which develops while chewing food. This is also called jaw claudication. Claudication develops because of inadequate blood flow to the muscles involved in chewing.
●Vision loss – Up to 20 percent of people with GCA can 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 GCA develop a dry cough or a sore throat.
●Thoracic aortic aneurysms – A thoracic aortic aneurysm occurs when the aorta, the main artery that carries blood from the heart, develops a weak spot and balloons outward. Aneurysms do not cause symptoms but can be life-threatening if they burst. While uncommon, this occurs somewhat more frequently in patients with GCA.
People with GCA can be tested for this type of aneurysm with a once-yearly chest radiograph, with echocardiography, or sometimes with other imaging techniques. (See "Management of thoracic aortic aneurysm in adults".)
Giant cell arteritis diagnosis — Your doctor or nurse will perform tests for GCA if you have some of the symptoms listed above. He or she will perform an exam and will 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), or you may need imaging tests (eg, radiograph, ultrasound, and magnetic resonance imaging [MRI] or positron emission tomography [PET] scanning).
Giant cell arteritis treatment
Glucocorticoid — Glucocorticoid (also called steroid) treatment in higher doses is usually recommended once the diagnosis of GCA is strongly suspected, even before it has been proven by biopsy.
If there is no evidence of arteritis on biopsy or on ultrasound and other imaging studies, and if your doctor is not convinced you have GCA, he or she will recommend stopping glucocorticoids (see "Treatment of giant cell (temporal) arteritis"). Sometimes glucocorticoid treatment is continued, even if there is no confirmation of arteritis, especially if your doctor strongly suspects that you have GCA.
Many people continue taking glucocorticoids for several months or up to one to two years. The dose of glucocorticoids is tapered slowly and carefully so that symptoms of GCA do not recur.
Other treatments — Other treatments for GCA have been tried, such as methotrexate, but these treatments are only used as supplements to glucocorticoids. Only glucocorticoids have been shown to be effective for the treatment of GCA and, in particular, for the prevention of loss of vision, but newer treatments are being actively investigated.
Polymyalgia rheumatica — In most people, polymyalgia rheumatica (PMR) tends to run its course over one to several years, and glucocorticoid therapy can eventually be stopped. In some patients, the disease can persist.
Giant cell arteritis — Giant cell arteritis (GCA) also tends to run its course over one to several years, though occasionally headache and other symptoms can reappear during the first few months after it has been diagnosed. But it is important to remember that if vision is intact when GCA is diagnosed, and if higher-dose, daily glucocorticoid treatment is started immediately, the risk of future vision loss is very small. The dose of glucocorticoids can eventually be reduced and then stopped in many if not most patients.
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 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.
Patient level information — UpToDate offers two types of patient education materials.
The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.
Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.
This topic currently has no corresponding Beyond the Basics content.
Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.
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 of thoracic aortic aneurysm in adults
The following organizations also provide reliable health information.
●American College of Rheumatology
●National Library of Medicine
All topics are updated as new information becomes available. Our peer review process typically takes one to six weeks depending on the issue.