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 1
to several years. With treatment, the symptoms of polymyalgia rheumatica
are quickly controlled, but relapse if treatment is stopped too
early.
Giant cell arteritis, also known as temporal arteritis and
cranial arteritis, is a disorder that results in swelling of arteries in
the head (most often the temporal arteries, which are located on the
temples on each side of the head), neck, and arms. This swelling causes
the arteries to narrow, reducing blood flow. Early treatment is critical
for good prognosis.
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 polymyalgia symptoms
disappear. About half of the people affected by 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 at onset
is 70 years. Polymyalgia rheumatica and giant cell arteritis are quite
common. In the United States, it is estimated that 700 per 100,000
people in the general population over 50 years of age develop
polymyalgia rheumatica. An estimated 200 per 100,000 people over the age
of 50 develop giant cell arteritis.
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. People are likely to experience headaches, pain in the temples,
and blurred or double vision. Pain may also affect the jaw and tongue.
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
forms of arthritis and/ or other rheumatic diseases. 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 can
be 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:
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 suspicion of giant cell arteritis.
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 1 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 corticosteroid medication,
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
-
myopathy (muscle wasting)
-
glaucoma
-
increased 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 productive, 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 looking for markers of the diseases,
exploring treatments, and studying why the two disorders often occur
together.
With funding from the National Eye Institute, a new mouse
model of giant cell arteritis is being used to examine interactions
between the immune system and blood vessels to explain tissue
damage.
Keeping on Top of Your Condition
Keeping in tune with your disease or condition not only makes treatment less intimidating but also increases its chance of success, and has been shown to lower a patients risk of complications. As well, as an informed patient, you are better able to discuss your condition and treatment options with your physician.
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