MEDICAL CONDITIONS
Polymyalgia Rheumatica (PMR)
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Giant Cell Arteritis (Temporal Arteritis) Medical Author: William C. Shiel Jr., MD, FACP, FACR
What is polymyalgia rheumatica?
Polymyalgia rheumatica (PMR) is a disorder of the muscles and joints of older persons. It is characterized by pain and stiffness, affecting both sides of the body, and involving the shoulders, arms, neck, and buttock areas. Patients with polymyalgia rheumatica are typically over the age of 50 years.
What causes polymyalgia rheumatica?
The cause of polymyalgia rheumatica is not known. Recent research has indicated that genetic (inherited) factors play a role in who becomes afflicted with the illness. Theories have included viral stimulation of the immune system in genetically susceptible individuals.
What are symptoms of polymyalgia rheumatica?
The onset of the illness can be sudden. A patient may have a healthy history until awakening one morning with stiffness and pain of muscles and joints throughout the body. These symptoms persist and are often accompanied by an intense sensation of fatigue. Some patients notice a gradual loss of appetite, weight, and energy. Depression can occur.
How is polymyalgia rheumatica diagnosed?
The doctor frequently notes muscle tenderness and that the motion of the shoulders is limited by pain. The joints are usually not swollen. However, swelling of the small joints of the hands, wrists, and/or knees can occur. Blood testing for inflammation is generally abnormal, as indicated by a significant elevation in the erythrocyte sedimentation rate (ESR) and/or C-reactive protein. There are no specific tests, however, for polymyalgia rheumatica and x-rays are normal. The diagnosis is based on the characteristic history of persisting muscle and joint pain and stiffness associated with the elevated ESR. It is also not unusual for patients to have slight elevations of liver blood tests .
How is polymyalgia rheumatica treated?
The treatment of polymyalgia rheumatica is directed toward reducing inflammation. While some patients with mild symptoms can improve with nonsteroid antiinflammatory drugs, such as aspirin or ibuprofen (Motrin, Advil), most patients respond best to low doses of cortisone medications, such as prednisone or prednisolone . Not infrequently, a single day's cortisone medication eases many of the symptoms! In fact, the rapid, gratifying results with low dose cortisone medications is characteristic of polymyalgia rheumatica.
The dose of prednisone is gradually reduced while the doctor monitors the symptoms and normalization of the blood ESR. Reactivation of symptoms can require periodic adjustments in the prednisone dosage. Most patients are able to completely wean from prednisone within several years. Some patients require longer term treatment. Occasionally, patients have a recurrence years after the symptoms have resolved. The ideal prednisone dosing regimen continues to be sought by researchers.
What is the prognosis for patients with polymyalgia rheumatica?
The prognosis for patients with isolated polymyalgia rheumatica is ultimately very good. Polymyalgia rheumatica can occur in association with giant cell arteritis (see below). One of the keys to successful treatment is gradual, and not rapid, tapering of the medications. This can avoid unwanted flare-ups of the disease.
Because the medications prednisone and prednisolone are associated with potential bone toxicity, causing osteoporosis , patients should consider calcium and vitamin D supplementation. Women should have bone mineral density testing and osteoporosis medications, such as estrogen, alendronate (Fosamax), and risedronate (Actonel) are considered.
What is giant cell arteritis?
Giant cell arteritis, also called temporal arteritis or cranial arteritis, is a serious disease characterized by inflammation of the walls of the blood vessels ( vasculitis ). The vessels affected are the arteries (hence the name "arteritis"). Giant cell arteritis occurs in 10-15% of patients with polymyalgia rheumatica. The age of affected patients is over 50 years of age, identical to polymyalgia rheumatica. The onset of giant cell arteritis may be years before, after, or without accompanying polymyalgia rheumatica. The cause of giant cell arteritis is not known. Recent research is looking into possible infectious causes and some information suggests a possible relationship to the microbe Chlamydia. This will require further studies for verification.
What are symptoms of giant cell arteritis?
Because giant cell arteritis is commonly due to inflammation of the arterial blood vessels affecting the head, it frequently leads to headaches, pain in the jaw when repetitively chewing, and tenderness of the scalp (usually over the inflamed arteries of the sides of the head (temporal area). It is also commonly associated with fatigue, low-grade fevers, and weight loss. The muscle aching of polymyalgia rheumatica is seen in nearly half of the patients with giant cell arteritis, either before, during, or after the onset of the the arteritis.
When the arteries affected by giant cell arteritis become inflamed they can narrow to the degree that the blood flow through them is limited. This can cause serious deficiency of oxygen supply to the tissues normally supplied by these arteries. Deficient oxygenation of the eyes or brain can lead to impaired or double vision, blindness, or stroke . Less commonly, inflammation of the blood vessels supplying the arms can lead to arm pain when the arms are used.
How is giant cell arteritis diagnosed?
The diagnosis of giant cell arteritis is suspected when a patient over the age of 50 (usually over 60) years of age develops symptoms above and/or suddenly develops blindness or stroke. The diagnosis is supported by signs of inflammation in the blood indicated by an elevation in the erythrocyte sedimentation rate (ESR) and/or C-reactive protein.
The diagnosis is confirmed with a biopsy of an artery, usually an artery on the side of the scalp called the temporal artery. This biopsy is performed under local anesthesia in an outpatient setting. It requires an incision into the scalp over the artery in the skin. The tissue of this area is supplied by blood from many vessels and does just fine after removal of the piece of the artery.
How is giant cell arteritis treated?
The goal of the treatment of giant cell arteritis is to suppress the inflammation within the arteries. When giant cell arteritis is diagnosed, high doses of cortisone medications, either intravenously or by mouth are necessary. The high doses required to quiet the inflamed arteries frequently are associated with side effects, including sweats, weight gain, bruising, puffiness of the face, erratic emotions, and others (For more information about the effects of cortisone medications, please read the prednisone article).
As in the treatment of polymyalgia rheumatica, because the cortisone medications are associated with potential bone toxicity, causing osteoporosis , patients should consider calcium and vitamin D supplementation. Women should have bone mineral density testing and osteoporosis medications, such as estrogen, alendronate (Fosamax), and risedronate (Actonel) are considered.
Recent studies have shown that low-dose aspirin can reduce the risk of stroke and visual loss in patients with giant cell arteritis.
What is the prognosis for patients with giant cell arteritis?
Giant cell arteritis usually runs a self-limited course, over months to years. Rarely, the inflammation may not respond to high doses of cortisone, and stronger medications that suppress the immune system have been tried, such as methotrexate . In most patients, the cortisone medications can gradually be tapered according to the symptoms while the ESR blood test is monitored.
The effects of the arteritis depend not only on the amount of inflammation in the arteries, but also on the location of the arteries in various body tissues. Recently, research scientists studying arteritis have discovered that the location of the inflamed arteries in the body and whether it is associated with polymyalgia rheumatica, seem to relate to different features of specialized white blood cells (T cells) that are infiltrating the artery walls. If these T cell differences can be further classified according to the patterns of inflammation in patients, this could lead to a new era of "customized" treatments for giant cell arteritis. - Polymyalgia rheumatica causes pain and stiffness in muscles and joints.
- Polymyalgia rheumatica is diagnosed by characteristic symptoms associated with abnormal blood testing for inflammation.
- Polymyalgia rheumatica is treated with low doses of cortisone medications.
- Giant cell arteritis is a result of inflammation of arteries.
- Giant cell arteritis can lead to blindness and/or stroke.
- Giant cell arteritis is detected by a biopsy of an artery.
- Giant cell arteritis is treated with high-dose cortisone medications.
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