Polymyalgia Rheumatica and Giant Cell Arteritis

Topic Overview

What are polymyalgia rheumatica and giant cell arteritis?

Polymyalgia rheumatica and giant cell arteritis are inflammatory problems.

In polymyalgia rheumatica (say "paw-lee-my-AL-juh roo-MAT-ih-kuh"), the joints and the areas around them get stiff and may ache. It mostly happens in the neck, shoulders, and hip.

In giant cell arteritis (say "ar-tuh-RY-tus"), inflammation occurs in the blood vessels that carry blood up through the neck to the head. It mostly affects the arteries that carry blood to the eyes, temple, and jaw. Giant cell arteritis is also called temporal arteritis.

Polymyalgia rheumatica and giant cell arteritis can occur at the same time and affect the same groups of people. Experts believe that the two problems are linked.

Polymyalgia rheumatica is more common. But giant cell arteritis is more dangerous, because it can cause loss of vision and may lead to a stroke or mini-strokes. People who have giant cell arteritis need treatment right away.

What causes polymyalgia rheumatica and giant cell arteritis?

Experts don't fully understand the cause of these conditions. It may be that the body's defense system (immune system) is attacking the body's own tissues. Your genes may play a role in this. For example, people whose ancestors are from Scandinavia or Northern Europe are more likely to have these problems.

What are the symptoms?

Symptoms of polymyalgia rheumatica often start suddenly and get worse without treatment. The problem can cause:

  • Muscle pain and morning stiffness in the neck, shoulders, and hip area.
  • Tiredness and lack of energy.
  • Fever.
  • Weight loss.

Symptoms of giant cell arteritis can start either suddenly or slowly. This problem needs treatment right away, because it can cause blindness, a stroke, or mini-strokes. Symptoms include:

  • A headache, often near the temple or around the eye.
  • Vision problems.
  • Tenderness of the temple or scalp.
  • Pain or aching of the tongue or jaw.

How are polymyalgia rheumatica and giant cell arteritis diagnosed?

Your doctor will do a physical exam and ask you about your symptoms and past health. He or she may suggest blood tests to find out if you have the conditions. Your doctor will have to rule out arthritis, because the symptoms are similar. To see if you have giant cell arteritis, a surgeon may take a sample (biopsy) from a blood vessel on your temple and test it for inflammation.

How are they treated?

Your doctor will give you steroid medicines to treat the inflammation. People often feel better in a day or two. Most of the time, symptoms improve quickly and go away 2 to 4 weeks after treatment begins. After this, most people need to continue to take steroid medicines for 1 to 2 years or sometimes longer. This helps to control symptoms and to prevent the problems from coming back.

Steroid medicines can cause your bones to thin (osteoporosis). Calcium is important for keeping your bones strong. So you need to make sure you are getting enough calcium, enough vitamin D, and enough weight-bearing exercise to strengthen your bones. Your doctor may also give you a medicine to prevent bone thinning.

Who gets polymyalgia rheumatica and giant cell arteritis?

These problems most often occur after the age of 50. And the risk increases after that as you get older.

Frequently Asked Questions

Learning about polymyalgia rheumatica and giant cell arteritis:

Being diagnosed:

Getting treatment:

Symptoms

Polymyalgia rheumatica

Symptoms of polymyalgia rheumatica (PMR) often occur suddenly and get worse without treatment. Typical polymyalgia rheumatica symptoms include pain and morning stiffness in the:

  • Shoulder.
  • Neck.
  • Pelvic and hip region.

These symptoms are caused by inflammation of joints, tendons, and bursae of the hip and shoulder regions. The pain affects both sides of the body. For example, both shoulders will usually be painful, not just one. Usually, both the shoulder and hip areas are affected.

Other symptoms of polymyalgia rheumatica may include:

  • Feeling tired and having no energy or strength (fatigue).
  • Weight loss.
  • Fever.
  • Depression.
  • Swelling of the knees, wrists, or ankles.

Giant cell arteritis

Symptoms of giant cell arteritis (GCA) can develop gradually or suddenly. They require immediate treatment.

Symptoms of giant cell arteritis may include:

  • Headache, often near the temple or around the eye (temporal headache). Headaches are a symptom for most people who are examined for giant cell arteritis. The headache may begin as a dull, throbbing pain on one side of the head around the eye or near the temple. Sometimes the pain may feel like a stabbing or burning sensation.
  • Vision problems. These problems may come on quickly and be temporary, but sometimes they do not go away. Examples include:
    • Brief loss of vision or partial loss. This can last seconds to minutes.
    • Blurry or double vision.
    • Blindness.
    • Patches of poor vision surrounded by normal vision.
    • Decreased sharpness (acuity) of vision or focus.
  • Tenderness on the side of the head (temple) or scalp. The blood vessel on the temple may also look swollen and its pulse may be decreased or absent. You may notice tenderness when you wear your glasses or comb your hair.
  • Pain, aching, weakness, or cramping (claudication) of the tongue or jaw, especially when you chew food or talk for long periods of time.

Other uncommon symptoms of giant cell arteritis may include cough, hoarseness, chest pain, and arm weakness or cramps.

Some people with giant cell arteritis do not have the typical symptoms of headache, jaw pain, and vision problems. About half of people with giant cell arteritis will also have symptoms of polymyalgia rheumatica.1

Untreated giant cell arteritis can cause narrowing (constriction) of some of the arteries in the skull or head, reducing blood flow. As a result, a person with untreated giant cell arteritis is at greater risk of blindness, stroke, or mini-strokes (transient ischemic attacks, or TIAs).

Exams and Tests

To diagnose polymyalgia rheumatica (PMR) and giant cell arteritis (GCA), your doctor will do a physical exam. He or she will ask you about your past health and symptoms. Because the two conditions often occur together, it is important that your doctor determine which one is causing your symptoms. Giant cell arteritis can be serious and needs immediate treatment with higher doses of medicine than polymyalgia rheumatica does.

Age is an important factor in making the diagnosis, because these conditions rarely occur in people younger than 50. A limited range of motion in the arms may be a sign of polymyalgia rheumatica. And arteries on the temple or forehead that are swollen, lumpy feeling, or tender may be a sign of giant cell arteritis.

Tests that can help diagnose polymyalgia rheumatica and giant cell arteritis include:

  • Erythrocyte sedimentation rate (ESR). This test measures how quickly red blood cells (erythrocytes) settle in a test tube in 1 hour. A high ESR may be a sign of polymyalgia rheumatica or giant cell arteritis.
  • Complete blood count (CBC). People with either polymyalgia rheumatica or giant cell arteritis often have mild anemia. A CBC can show this.
  • C-reactive protein. This test measures the amount of C-reactive protein (CRP) produced when you have inflammation somewhere in your body. Both giant cell arteritis and polymyalgia rheumatica can cause a high CRP level.
  • Temporal artery biopsy. When your doctor suspects giant cell arteritis, this test can confirm the condition. In this biopsy, a surgeon will take a sample of a blood vessel on your temple and test it for inflammation.
  • Ultrasound. In some cases, ultrasound of the arteries on the side of the face near the temple (temporal arteries) may help diagnose giant cell arteritis. This way of using ultrasound is still being studied for use in the United States.

Your doctor may confirm a diagnosis of polymyalgia rheumatica with a trial of corticosteroid medicine. If you have polymyalgia rheumatica, you are very likely to have great relief of symptoms within 2 to 4 days of starting treatment.

Tests that may be done to be sure another condition is not causing your symptoms include:

Treatment Overview

Corticosteroid medicine is the most common treatment for both polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). People with either or both conditions usually have fast relief of their symptoms soon after starting corticosteroid treatment.

Initial treatment

Corticosteroid medicine treats both polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). In general, you will use the corticosteroids at a certain dose until your symptoms go away and your lab tests are normal. When this occurs, your doctor will gradually begin reducing your medicine.

For polymyalgia rheumatica
  • You take lower doses of corticosteroids than for giant cell arteritis.
  • You are likely to have a lot of pain relief in a few days. Symptoms usually go away, and your lab results are normal in 2 to 3 weeks. If your symptoms don't start going away in 2 to 4 days, your doctor may give you a higher dose and do further testing to be sure your condition is polymyalgia rheumatica.
  • After your symptoms go away and your lab tests are normal, your doctor will gradually lower your dose, about every few weeks to months.
  • You will probably have to take corticosteroids for about 2 years or more to prevent the symptoms from coming back (relapse). Some people only need to take the medicine for less than a year.
For giant cell arteritis
  • You take higher doses of corticosteroids than used in polymyalgia rheumatica.
  • Because giant cell arteritis can cause serious complications, such as stroke or blindness, your doctor may start treatment immediately, even if the diagnosis has not been confirmed by tests.
  • You are likely to have a lot of pain relief in a few days. Symptoms usually go away, and your lab results are normal in 2 to 4 weeks. If your symptoms don't start to go away, your doctor may give you a higher dose.
  • After your symptoms go away and your lab tests are normal, your doctor will gradually lower your dose, about every 2 to 4 weeks in the first several months, then less often after that.
  • You will probably have to take corticosteroids for 1 to 2 years to prevent relapse, although many people with the condition need them for longer than 2 years.

How long you need to take corticosteroids depends on how severe your condition is, whether it appears cured or not (remission), and how often you have relapses. Some people are not able to completely stop taking corticosteroids. If this happens, your doctor may suggest another medicine such as methotrexate to help control your symptoms and keep the condition from coming back.

Ongoing treatment

After your symptoms have gone away and your lab tests are normal, ongoing treatment for polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) usually includes continued use of corticosteroid medicine.

Your doctor gradually lowers the amount of medicine you take. How quickly he or she does this varies with each person.

  • In polymyalgia rheumatica, most people have to take corticosteroids for about 2 years.
  • In giant cell arteritis, most people have to take corticosteroids for 1 to 2 years. But many people with the condition need them for longer than 2 years.

If your symptoms do not improve with corticosteroid medicine, your doctor may need to test you for other conditions that are similar to polymyalgia rheumatica or giant cell arteritis.

Your doctor will track your condition while you are taking corticosteroid medicine and for 6 to 12 months after you stop taking the medicine. This may include regular appointments or phone calls to discuss your symptoms. And you may need tests to measure your erythrocyte sedimentation rate (ESR) or your C-reactive protein (CRP) value.

If you need long-term corticosteroid treatment for either condition, you are at risk for bone thinning (osteoporosis). This is because corticosteroids reduce how well your body takes in calcium, which is important in building strong bones. Your doctor may recommend a bone density (DXA) test to see if you need medicine (bisphosphonates) to prevent osteoporosis. Or he or she may simply start you on bisphosphonates without the test. For more information, see the topic Osteoporosis.

Treatment if the conditions get worse

In both polymyalgia rheumatica (PMR) and giant cell arteritis (GCA), your symptoms may come back (relapse) after a period of improvement. This often occurs in the first 2 years of treatment or during the first 12 months after you stop taking corticosteroid medicine. A relapse usually occurs if the dose of corticosteroids is reduced or withdrawn too quickly. But sometimes people have a relapse over the first 2 years that is not related to how corticosteroids are used. If you have a relapse of symptoms, your doctor will increase the corticosteroid dosage for a period of time then gradually decrease it after your symptoms are gone.

In rare cases, giant cell arteritis can affect the main part of the aorta in the chest, rather than one of its smaller branches, causing an aortic aneurysm. If this were to happen, you would feel severe chest pain that could extend to your back, and you could faint or have symptoms of a stroke. If you have giant cell arteritis and have such symptoms, contact your doctor or call 911 immediately. Though rare, such an event may be life-threatening. Your doctor may recommend that you have an annual chest X-ray to watch for an aortic aneurysm.

Giant cell arteritis can lead to partial or complete loss of vision in one or both eyes. If you have sudden onset of double, blurred, or "browned-out" vision, or temporary loss of vision, your doctor may increase your corticosteroid dose. And for a few days he or she may give you the dose directly into a blood vessel (intravenously) to try to prevent permanent or further loss of vision.

What to think about

If you have polymyalgia rheumatica and do not have giant cell arteritis, your doctor may suggest using nonsteroidal anti-inflammatory drugs (NSAIDs) when you are lowering the dose of your corticosteroid medicine. Always talk to your doctor or nurse before using these medicines. Be safe with medicines. Read and follow all instructions on the label.

Some doctors recommend taking aspirin if you have giant cell arteritis. This is because some studies show that doing so may reduce the risk of vision loss, stroke, or transient ischemic attacks (blood flow to the brain is interrupted).2

Corticosteroids can cause serious side effects, including high blood pressure (hypertension) and bone thinning (osteoporosis). If you are taking long-term corticosteroids to treat polymyalgia rheumatica or giant cell arteritis:

  • You will need to get enough calcium and vitamin D.
  • You may also need bisphosphonate medicine such as alendronate or risedronate to prevent bone loss.
  • You can help reduce the risk of osteoporosis by lifting weights and doing weight-bearing exercise such as walking. For more information, see the topic Osteoporosis.
  • Your doctor may suggest that you take a proton pump inhibitor such as Prilosec or a medicine called an H2 blocker (acid reducer) to protect your digestive (gastrointestinal or GI) tract, including your esophagus and stomach. Taking a lot of medicine for a long period of time can irritate these areas and can even lead to ulcers. Proton pump inhibitors and H2 blockers decrease the amount of acid the stomach makes. This reduces irritation of the stomach and can help reduce irritation of the esophagus.

Methotrexate, or other medicines that suppress your immune system, may be used for polymyalgia rheumatica or giant cell arteritis alone or with corticosteroids to reduce the corticosteroid dose and limit its side effects. Research results are mixed on how well this treatment works.3

Giant cell arteritis sometimes affects the large arteries of the arm. In rare cases, this interferes with blood flow. This can cause pain and cramping when you use your arm. In a small study, balloon angioplasty opened up the arteries of the arm to reduce symptoms of giant cell arteritis.4

Home Treatment

To feel your best during treatment for polymyalgia rheumatica (PMR) or giant cell arteritis (GCA):

  • Take your medicine as prescribed by your doctor.
  • Work closely with your doctor and nurse. Be sure to report medicine side effects or any symptoms of giant cell arteritis, such as headache, jaw pain, and vision problems.
  • Eat a balanced diet.
  • Stop smoking, and avoid being around tobacco smoke.
  • Reduce how much alcohol you drink. It's a good idea to have no more than one beer or one glass of wine each day.
  • Be sure you get enough calcium and vitamin D to help prevent bone thinning (osteoporosis) if you are taking corticosteroids.
  • Get mild, regular weight-bearing exercise. This will help keep your bones strong and bolster your physical and emotional ability to handle your illness.

If any of your symptoms get worse or come back, contact your doctor or nurse.

Other Places To Get Help

Organizations

American College of Rheumatology
2200 Lake Boulevard NE
Atlanta, GA  30319
Phone: (404) 633-3777
Fax: (404) 633-1870
Web Address: www.rheumatology.org
 

The American College of Rheumatology (ACR) and the Association of Rheumatology Health Professionals (ARHP, a division of ACR) are professional organizations of rheumatologists and associated health professionals who are dedicated to healing, preventing disability from, and curing the many types of arthritis and related disabling and sometimes fatal disorders of the joints, muscles, and bones. Members of the ACR are physicians; members of the ARHP include research scientists, nurses, physical and occupational therapists, psychologists, and social workers. Both the ACR and the ARHP provide professional education for their members.

The ACR website offers patient information fact sheets about rheumatic diseases, about medicines used to treat rheumatic diseases, and about care professionals.


National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health
1 AMS Circle
Bethesda, MD  20892-3675
Phone: 1-877-22-NIAMS (1-877-226-4267) toll-free
Phone: (301) 495-4484
Fax: (301) 718-6366
TDD: (301) 565-2966
Email: niamsinfo@mail.nih.gov
Web Address: www.niams.nih.gov
 

The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) is a governmental institute that serves the public and health professionals by providing information, locating other information sources, and participating in a national federal database of health information. NIAMS supports research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases and supports the training of scientists to carry out this research.

The NIAMS website provides health information referrals to the NIAMS Clearinghouse, which has information packages about diseases.


References

Citations

  1. Hellmann DB, Imboden JB Jr (2012). Musculoskeletal and immunologic disorders. In SJ McPhee, MA Papadakis, eds., 2012 Current Medical Diagnosis and Treatment, 51st ed., pp. 787–846. New York: McGraw-Hill.
  2. Spiera RF, Paget SA (2012). Polymyalgia rheumatica and temporal arteritis. In L Goldman, A Shafer, eds. Goldman's Cecil Medicine, 24th ed., pp. 1728–1731. Philadelphia: Saunders.
  3. Hellmann DB (2013). Giant cell arteritis, polymyalgia rheumatica, and Takayasu's arteritis. In GS Firestein et al., eds., Kelley's Textbook of Rheumatology, 9th ed., vol. 2, pp. 1461–1480. Philadelphia: Saunders.
  4. Both M, et al. (2006). Balloon angioplasty of arteries of the upper extremities in patients with extracranial giant-cell arteritis. Annals of the Rheumatic Diseases, 65(9): 1124–1130.

Credits

By Healthwise Staff
Adam Husney, MD - Family Medicine
Anne C. Poinier, MD - Internal Medicine
Last Revised April 10, 2013

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