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POLYMYALGIA
RHEUMATICA
AND
GIANT CELL ARTERITIS
What Are Polymyalgia
Rheumatica and Giant Cell Arteritis?
Polymyalgia rheumatica
is a rheumatic disorder that is associated with moderate to
severe muscle pain and stiffness in the neck, shoulder, and
hip area. Stiffness is most noticeable in the morning. This
disorder may develop rapidly--in some patients overnight. In
other people, polymyalgia rheumatica develops more gradually.
The cause of polymyalgia rheumatica is not known; however, possibilities
include immune system
abnormalities and genetic factors. The fact that polymyalgia
rheumatica is rare in people under the age of 50 suggests it
may be linked to the aging process.
Polymyalgia rheumatica
may go away without treatment in one to several years. But with
treatment, the symptoms of polymyalgia rheumatica disappear
quickly.
Giant cell arteritis,
also known as temporal arteritis and cranial arteritis, is a
disorder that results in swelling of arteriesin
the head (most often the temporal arteries), neck, and arms.
This swelling causes the arteries to narrow, reducing blood
flow. Giant cell arteritis should be treated as soon as it is
detected to prevent blindness.
How Are Polymyalgia
Rheumatica and Giant Cell Arteritis Related?
It is unclear how
or why polymyalgia rheumatica and giant cell arteritis are related,
but an estimated 15 percent of people in the United States with
polymyalgia rheumatica also develop giant cell arteritis. Patients
can develop giant cell arteritis either at the same time as
polymyalgia rheumatica, or after the symptoms disappear. About
half of the people suffering from giant cell arteritis also
have polymyalgia rheumatica.
When a person is
diagnosed with polymyalgia rheumatica, the doctor also should
look for symptoms of giant cell arteritis because of the risk
of blindness. With proper treatment, the disease is not threatening.
Untreated, however, giant cell arteritis can lead to serious
complications including permanent vision loss and stroke. Patients
must learn to recognize the signs of giant cell arteritis, because
they can develop even after the symptoms of polymyalgia rheumatica
disappear. Patients should report any symptoms to the doctor
immediately.
Who Is at Risk?
White women over
the age of 50 are most at risk of developing polymyalgia rheumatica
and giant cell arteritis. Women are twice as likely as men to
develop the conditions. Both conditions almost exclusively affect
people over the age of 50. The average age of onset is 70 years.
Polymyalgia rheumatica and giant cell arteritis are quite common.
In the United States, it is estimated that 500 per 100,000 people
in the general population over 50 years of age develop polymyalgia
rheumatica.
What Are the Symptoms?
The primary symptoms
of polymyalgia rheumatica are moderate to severe stiffness and
muscle pain near the neck, shoulders, or hips. The stiffness
is more severe upon waking or after a period of inactivity,
and typically lasts longer than 30 minutes. People with this
condition also may have flu-like symptoms, including fever,
weakness, and weight loss.
Early symptoms of
giant cell arteritis also may resemble the flu. As the condition
progresses, people are likely to experience headaches, pain
in the temples, and blurred or double vision. Pain may also
affect the jaw and tongue. Doctors suspect giant cell arteritis
in people with these symptoms who previously were diagnosed
with polymyalgia rheumatica.
How Are Polymyalgia
Rheumatica and Giant Cell Arteritis Diagnosed?
No single test is
available to definitively diagnose polymyalgia rheumatica. To
diagnose the condition, a physician considers the patient's
medical history, including symptoms that the patient reports
and results of laboratory tests that can rule out other possible
diagnoses.
The most typical
laboratory finding in people with polymyalgia rheumatica is
an elevated erythrocyte sedimentation rate, commonly referred
to as the sed rate. This test measures how quickly red blood
cells fall to the bottom of a test tube of unclotted blood.
Rapidly descending cells (an elevated sed rate) indicate inflammation
in the body. While the sed rate measurement is a helpful diagnostic
tool, it alone does not confirm polymyalgia rheumatica. An abnormal
result indicates only that tissue is inflamed, which also is
a symptom of many other types of arthritis. Before making a
diagnosis of polymyalgia rheumatica, the doctor may perform
additional tests to rule out other conditions, including rheumatoid
arthritis, because symptoms of polymyalgia rheumatica and rheumatoid
arthritis are very similar.
The doctor may recommend
a test for rheumatoid
factor (RF). RF is an antibody
sometimes found in the blood. (An antibody is a special protein
made by the immune system.) People with rheumatoid arthritis
are likely to have RF in their blood, but most people with polymyalgia
rheumatica do not. If the diagnosis still is unclear, a physician
may conduct additional tests to rule out other disorders.
Doctors and patients
both need to be aware of the risk of giant cell arteritis in
people with polymyalgia rheumatica and should be on the lookout
for symptoms of the disorder. Severe headaches, jaw pain, and
vision problems are typical symptoms of giant cell arteritis.
In addition, physical examination may reveal an abnormal temporal
artery (located on the temples on each side of the head)--tender
to the touch, inflamed, and with reduced pulse. Because of the
possibility of permanent blindness, a temporal artery biopsy
is recommended if there is any doubt about the diagnosis. In
a person with giant cell arteritis, the biopsy will show abnormal
cells in the artery walls. Some patients showing symptoms of
giant cell arteritis will have negative biopsy results. In such
cases the doctor may suggest a second biopsy.
What Are the Treatments?
Polymyalgia rheumatica
usually disappears without treatment in one to several years.
With treatment, however, symptoms disappear quickly, usually
in 24 to 48 hours. If there is no improvement, the doctor is
likely to consider other possible diagnoses.
The treatment of
choice is corticosteroidmedication,
usually prednisone. Polymyalgia rheumatica responds to a low
daily dose of prednisone. The dose is increased as needed until
symptoms disappear. Once symptoms disappear, the doctor may
gradually reduce the dosage to determine the lowest amount needed
to alleviate symptoms. The amount of time that treatment is
needed is different for each patient. Most patients can discontinue
medication after 6 months to 2 years. If symptoms recur, prednisone
treatment is required again.
Nonsteroidal
anti-inflammatory drugs (NSAIDs) such as aspirin and ibuprofen
also may be used to treat polymyalgia rheumatica. The medication
must be taken daily and long-term use may cause stomach irritation.
For most patients, NSAIDs alone are not enough to relieve symptoms.
Giant cell arteritis
carries a small but definite risk of blindness. The blindness
is permanent once it happens. A high dose of prednisone is needed
to prevent blindness and should be started as soon as possible,
perhaps even before the diagnosis is confirmed with a temporal
artery biopsy. When treated, symptoms quickly disappear. Typically,
people with giant cell arteritis must continue taking a high
dose of prednisone for 1 month. Once symptoms disappear and
the sed rate is normal and there is no longer a risk of blindness,
the doctor can begin to gradually reduce the dose. When treated
properly, giant cell arteritis rarely recurs.
People taking low
doses of prednisone rarely experience side effects. Side effects
are more common among people taking higher doses. But all patients
should be aware of potential effects, which include:
- fluid retention
and weight gain
- rounding of the
face
- delayed wound
healing
- bruising easily
- diabetes
- glaucoma
- increase in blood
pressure
- decreased calcium
absorption in the bones, which can lead to osteoporosis
- irritation of
the stomach
People taking corticosteroids
may have some side effects or none at all. A patient should
report any side effects to the doctor. When the medication is
stopped, the side effects disappear. Because prednisone and
other corticosteroid drugs change the body's natural production
of corticosteroid hormones, the patient should not stop taking
the medication unless instructed by the doctor. The patient
and doctor must work together to gradually reduce the medication.
What Is the Outlook?
Most people with
polymyalgia rheumatica and giant cell arteritis lead normal,
active lives. The duration of drug treatment differs by patient.
Once treatment is discontinued, polymyalgia may recur; but once
again, symptoms respond rapidly to prednisone. When properly
treated, giant cell arteritis rarely recurs.
What Research Is
Being Conducted To Help People Who Have Polymyalgia Rheumatica
and Giant Cell Arteritis?
Researchers studying
possible causes of polymyalgia rheumatica and giant cell arteritis
are investigating the role of genetic predisposition,
immune system abnormalities, and environmental factors. Scientists
also are studying why the two disorders often occur together
and are exploring other treatments. Preliminary findings suggest
that low doses of methotrexate,
a drug used to treat some other rheumatic disorders, controls
polymyalgia rheumatica and maybe an effective treatment for
giant cell arteritis.
Where Can People
Get More Information About Polymyalgia Rheumatica and Giant
Cell Arteritis?
- Arthritis Foundation
1330 West Peachtree Street
Atlanta, Georgia 30309
404/872-7100
800/283-7800 or call your local chapter (listed in your local
telephone directory)
World Wide Web address: http://www.arthritis.org
Acknowledgments
The NIAMS gratefully
acknowledges the assistance of Gene G. Hunder, M.D., of the
Mayo Clinic, and Louis A. Healey, M.D. (retired), in the preparation
and review of this fact sheet.
The National
Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse (NAMSIC) is a public service sponsored by
the NIAMS that provides health information and information sources.
The NIAMS, a part of the National Institutes of Health (NIH),
leads the Federal medical research effort in arthritis and musculoskeletal
and skin diseases. The NIAMS sponsors research and research
training throughout the United States as well as on the NIH
campus in Bethesda, MD, and disseminates health and research
information.
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