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Polymyalgia rheumatica
By Mayo Clinic staffOriginal Article: http://www.mayoclinic.com/health/polymyalgia-rheumatica/DS00441
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Definition
Polymyalgia rheumatica is an inflammatory disorder that causes muscle pain and stiffness, primarily in your neck, shoulders, upper arms, hips and thighs. Symptoms of polymyalgia rheumatica (pol-ee-my-AL-juh roo-MA-ti-kah) usually begin quickly over a few days.
Most people who develop polymyalgia rheumatica are older than 65, and it rarely affects people younger than 50.
Anti-inflammatory drugs called corticosteroids improve the symptoms of polymyalgia rheumatica, but these drugs require careful monitoring for serious side effects.
Polymyalgia rheumatica is related to and may coexist with another inflammatory disorder called giant cell arteritis, which can cause headaches, visual impairment, jaw pain and other symptoms.
Symptoms
The signs and symptoms of polymyalgia rheumatica include:
- Aches or pain in your shoulders (often the first symptom)
- Aches or pain in your neck, lower back, buttocks, hips or thighs
- Tenderness in your upper arms
- Stiffness in affected areas, particularly in the morning or after prolonged inactivity, such as a long car ride
- Limited range of motion in affected areas
- Pain or stiffness in wrists or knees (less common)
When pain and stiffness first appear, it's common to have more general signs and symptoms, including:
- Low-grade fever
- Fatigue
- A general feeling of not being well (malaise)
- Loss of appetite
- Unintended weight loss
- Depression
When to see a doctor
See your doctor if you experience aches, pains or stiffness that:
- Is new
- Disrupts your sleep
- Limits your ability to do activities of daily living
Causes
The cause of polymyalgia rheumatica is not well understood. The pain and stiffness result from the activity of inflammatory cells and proteins that are normally a part of your body's disease-fighting immune system.
Inflammation is usually a response to injury or disease, but in some disorders — known collectively as rheumatic diseases — inflammatory activity occurs when there is no apparent need for the response.
In polymyalgia rheumatica, the inflammatory activity seems to be concentrated in tissues surrounding the affected joints. The muscle pain associated with the disorder is called referred pain, or pain that extends from joints to nearby muscles or other tissues.
Multiple causes
Research suggests that a combination of inherited (genetic) factors and external (environmental) factors may contribute to the onset of disease.
- Genetic factors. A gene or genes — not yet identified — may make a person more likely to develop polymyalgia rheumatica. The prevalence of the disorder among people of Northern European ancestry, patterns of family history of the disease and evidence from some genetic studies suggest the influence of inheritance.
- Environmental factors. Certain features of the disorder suggest that an infectious disease may be a contributing factor. For example, the sudden onset of symptoms would be expected from an infectious agent. Also, new cases often appear in cycles in the general population, a factor that would be consistent with the normal cycle of contagious viral diseases. Although studies have produced inconclusive results, several relatively common viral infections have been identified as possible triggers for polymyalgia rheumatica.
Giant cell arteritis
The inflammation-promoting cells and proteins that are active in polymyalgia rheumatica are also active in a disease known as giant cell arteritis. This disorder results in inflammation in the lining of arteries, most often the arteries located in the temples. Giant cell arteritis usually causes headaches, jaw pain, vision impairment and scalp tenderness. It can lead to permanent vision loss.
Polymyalgia rheumatica and giant cell arteritis may, in fact, be variations of the same disease. Studies have produced varying results, but the overlap of diagnoses is significant:
- As many as 20 percent of people with polymyalgia rheumatica may have giant cell arteritis.
- As many as 60 percent of the people with giant cell arteritis may have polymyalgia rheumatica.
Risk factors
Risk factors for polymyalgia rheumatica include:
- Age. Polymyalgia rheumatica affects older adults almost exclusively. The average age at onset of the disease is 70.
- Sex. Women are about two times more likely to develop the disorder.
- Ethnicity. People of Northern European origin — and Scandinavian descent, in particular — are more likely to have polymyalgia rheumatica than are people of other ethnicities.
Complications
Symptoms of polymyalgia rheumatica can greatly affect a person's ability to perform everyday activities. The pain and stiffness may contribute to difficulties with the following tasks:
- Getting out of bed, standing up from a chair or getting out of a car
- Bathing, combing your hair or performing other tasks related to personal hygiene
- Getting dressed or putting on a coat
These complications can affect a person's health, social interactions, physical activity and general well-being.
Preparing for your appointment
If you're experiencing aches, pains or stiffness in joints or muscles, you're likely to see your primary care doctor first. You may be referred to a specialist in inflammatory disorders of muscles and the skeletal system (rheumatologist).
You may want to bring a friend or relative to your appointment. This person, in addition to offering support, can write down information from your doctor or other clinic staff during the appointment.
What to expect from your doctor
Your doctor will likely ask you a number of questions. Be prepared to answer the following:
- Where is the pain or stiffness located?
- When did the symptoms begin?
- How would you rate your current level of pain on a scale of 1 to 10?
- Are symptoms worse at certain times of day or night?
- How long does stiffness last after you wake in the morning or after a long period of inactivity?
- Does the pain or stiffness limit your activities? Are you avoiding any activities because of the symptoms?
- Have you experienced similar episodes of pain or stiffness in the past? Was the condition diagnosed and treated?
- Have you experienced any new or severe headaches?
- Have you noticed any changes in your vision?
- Have you experienced any jaw pain?
Prepare a list
Make a list to share with your primary doctor or rheumatologist. This list should include:
- The name and contact information of any doctor you have seen recently or see regularly
- Prescription medications and dosages
- Over-the-counter drugs or dietary supplements you take regularly
Tests and diagnosis
Your answers to questions, a general physical exam and the results of tests can help your doctor determine the cause of pain and stiffness.
Exam
Your doctor will conduct an exam to assess your health in general, identify possible causes or rule out certain diseases. He or she may gently move your head and limbs to judge how much your symptoms affect your range of motion.
Blood tests
A nurse or assistant will draw a sample of your blood. This sample will be used for several laboratory tests that your doctor will order.
Some tests will screen for a number of factors, such as viral infections or changes in certain hormone levels, that can rule out certain conditions or lead to a diagnosis other than polymyalgia rheumatica. Other tests will assess the profile of various blood components that can indicate inflammatory activity or chronic disease in your body.
Test results particularly relevant to making a diagnosis of polymyalgia rheumatica include the following:
- Sed rate, or erythrocyte sedimentation rate (ESR), measures the distance red blood cells, or erythrocytes (uh-RITH-roh-sites), fall in a test tube in one hour. The distance indirectly measures the level of inflammation — the farther the red blood cells have descended, the greater the inflammatory response of your immune system. An increased rate occurs because of certain changes to red blood cell properties in response to inflammation.
- C-reactive protein test measures the concentration of C-reactive proteins in your blood. Production of this protein and its release into your bloodstream increase when your immune system initiates an inflammatory response. Therefore, a high concentration of C-reactive protein indicates increased inflammatory activity.
Imaging tests
Ultrasound imaging, which uses sound waves to produce images of soft tissues, may reveal inflammation of tissues within the shoulder and hip joints that can support a diagnosis of polymyalgia rheumatica. These images may also help identify or rule out other causes of your symptoms.
Monitoring for giant cell arteritis
Your doctor will monitor you for signs or symptoms that may indicate the onset of giant cell arteritis. Talk to your doctor immediately if you experience any of the following symptoms:
- New, unusual or persistent headaches
- Jaw pain or tenderness
- Blurred or double vision
- Scalp tenderness
If your doctor suspects a diagnosis of giant cell arteritis, he or she will order a biopsy of the artery in one of your temples. This procedure, performed with local anesthesia, removes a tiny sample of the artery, which is then examined in a laboratory for signs of inflammation.
Treatments and drugs
Corticosteroids
Polymyalgia rheumatica is usually treated with a low dose of an oral corticosteroid, such as prednisone. A daily dose at the beginning of the treatment is usually 10 to 20 milligrams a day.
Relief from pain and stiffness should occur within the first two or three days. If you're not feeling better in a few days, it's likely you don't have polymyalgia rheumatica. In fact, your response to medication is one way your doctor can confirm the diagnosis.
After the first two to four weeks of treatment, your doctor will gradually decrease your dosage depending on your symptoms and the results of sed rate and C-reactive protein tests. The goal is to keep you on as low a dose as possible. Most people with polymyalgia rheumatica need to continue the corticosteroid treatment for two to three years.
You will have frequent follow-up visits to monitor the treatment effect and assess for possible side effects.
Monitoring side effects
Long-term use of corticosteroids can result in a number of serious side effects. Your doctor will monitor you closely for potential problems. He or she may adjust your dosage and prescribe treatments to manage these reactions to corticosteroid treatment. Side effects include:
- Osteoporosis, the loss of bone density and weakening of bones, which increase the risk of bone fractures
- High blood pressure (hypertension), which increases the risk of cardiovascular disease
- High cholesterol, which also increases the risk of cardiovascular disease
- Diabetes, chronic high levels of blood sugar that can cause tissue damage in a number of body systems
- Cataracts, a clouding of the lenses of your eyes that can significantly impair your vision
- Depression or other disturbances in your emotional well-being
Calcium and vitamin D supplements
Your doctor will likely prescribe daily doses of calcium and vitamin D supplements to help prevent osteoporosis induced by corticosteroid treatment. The American Academy of Rheumatology recommends, at a minimum, the following daily doses for anyone taking corticosteroids for more than three months:
- 1,000 to 1,200 milligrams (mg) of calcium supplements
- Up to 1,000 international units (IUs) of vitamin D supplements
Physical therapy
Your doctor may recommend physical therapy to help you regain strength, coordination and your ability to perform everyday tasks after a long period of limited activity that polymyalgia rheumatica often causes.
Lifestyle and home remedies
Healthy lifestyle choices can help you manage the side effects that may result from corticosteroid treatment:
- Eat a healthy diet. Eat a diet of fruits, vegetables, whole grains, and low-fat meat and dairy products. Avoid foods with high levels of refined sugars and salt (sodium).
- Exercise regularly. Talk to your doctor about exercise that is appropriate for you to maintain a healthy weight and to strengthen bones and muscles.
- Use assistive devices. Use luggage and grocery carts, reaching aids, shower grab bars and other assistive devices to help make daily tasks easier. Wear low-heeled shoes to minimize the risk of falls. Talk to your doctor about whether the use of a cane or other walking aid is appropriate for you to prevent falls or other injury.
- Michet CJ, et al. Polymyalgia rheumatica. BMJ. 2008;336:765.
- Unwin B, et al. Polymyalgia rheumatica and giant cell arteritis. American Family Physician. 2006;74:1547.
- Polymyalgia rheumatica. American College of Rheumatology. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/polymyalgiarheumatica.asp. Accessed April 14, 2010.
- Polymyalgia rheumatica. The Merck Manuals: The Merck Manual of Healthcare Professionals. http://www.merck.com/mmpe/sec04/ch033/ch033i.html. Accessed April 6, 2010.
- Salvarani C, et al. Polymyalgia rheumatica and giant-cell arteritis. Lancet. 2008;372:234.
- Giant cell arteritis. American College of Rheumatology. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/giantcellarteritis.asp. Accessed April 15, 2010.
- Glucocorticosteroid-induced osteoporosis. American College of Rheumatology. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/gi-osteoporosis.asp. Accessed April 15, 2010.
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