Polymyalgia Rheumatica

Basics of polymyalgia rheumatica

Polymyalgia rheumatica (PMR) is a disorder that causes stiffness and aching that begins in the neck, shoulder and hip areas. It is not known whether it is a disease of the joints muscles or arteries. However it is thought that inflammation in these areas leads to pain and stiffness.

Incidence

Polymyalgia rheumatica typically affects people in their 60s and 70s. It is rare before age 50. It affects people of Northern European ancestry, particularly those from Scandinavia. It is rare among people of African ancestry.

PMR does not have strong hereditary tendencies.

Click to Enlarge

Symptoms

Polymyalgia rheumatica (PMR) typically starts very suddenly. People with PMR may be perfectly well one day and then feel the full effects of this disease the next day. PMR causes stiffness and aching of the muscles about the neck shoulders and hips (see figure 1). Most people with PMR have symptoms in at least two of these three areas. The shoulder region includes the muscles of the upper arm. The hip region includes the muscles of the lower back and thighs.

Stiffness is a major feature of PMR. It is worst first thing in the morning and when it is severe people complain that they have to roll themselves out of bed. Pain typically wakes people at night and turning over in bed may be difficult. The stiffness may be worse during periods of inactivity such as after a long car ride.

PMR may cause other symptoms. These include fatigue, weight loss and a slight fever. Joints in other areas of the body may ache as well. Sometimes joints in the hands swell. Numbness and tingling in the fingers (carpal tunnel syndrome) also may occur with PMR.

Most persons with this disease have been in good health before their first symptoms. People often do not understand why they feel so terrible when the disease begins. These symptoms usually make them feel very different than before and can be quite overwhelming because of their effect on everyday life.

PMR usually is not associated with any other disease. However, a person with PMR also may develop other forms of arthritis. Some people with PMR also have a condition called giant cell arteritis which is described later.

Diagnosis

There is no single test that can be used to make the diagnosis of PMR in all people.

PMR is a syndrome which is a collection of symptoms. This can make the diagnosis of PMR difficult. To make the diagnosis your doctor will consider your symptoms along with results of your physical examination and laboratory tests. Other rheumatic diseases infections and cancer can cause symptoms similar to PMR. It can take a while to finally make the diagnosis of PMR.

To find out if you have PMR your doctor will:

• ask about your symptoms and recent changes in your health
• perform a physical examination
• obtain certain blood tests

These blood tests will be used to check for diseases that cause symptoms similar to PMR such as rheumatoid arthritis. A blood test called the erythrocyte sedimentation (ESR) will likely be included. The ESR can measure the amount of inflammation present. This test measures how fast red blood cells cling together, fall, and settle toward the bottom of a glass tube like sediment in an hour's time. When inflammation is present the red blood cells sink more quickly and the ESR is higher than normal. As inflammation responds to medication the ESR usually goes down. In almost all persons with PMR the ESR is higher than normal. However, the ESR is higher than normal in other diseases besides PMR. As a result the diagnosis of PMR cannot be made by a blood test alone because the blood test cannot distinguish PMR from other conditions such as infection or other forms of arthritis.

Treatment

Treatment includes medications to help reduce inflammation as well as proper exercise and rest for some people in order to maintain joint flexibility muscle strength and function.

The goal in treating PMR is to help relieve pain, stiffness and achiness.

Medications

Corticosteroid drugs, strong medications that help reduce inflammation, are the usual medications used to treat PMR. They also help relieve stiffness and achiness. They act quickly in PMR. Most people feel better within a few days or sometimes even the next day. There are many forms of corticosteroids. For example, prednisone is a medication often used in PMR.

If your doctor places you on a corticosteroid you should follow instructions carefully. Your doctor may have you take the entire dose in the morning or have you divide the dose throughout the day. You probably will feel better very soon after starting this medicine. It is important to keep taking the medicine even though you are feeling better. PMR can return if you stop this medicine too quickly. The usual dose of prednisone is between 10 and 20 mg per day.

You may need to take corticosteroids as briefly as six months or as long as one or two years. Some people may need to be treated even longer. As you improve while on this medicine it is likely that the dose will slowly be decreased. Follow your doctor's instructions on how often to take your medication. Do not try to cut back the dose on your own or suddenly stop taking your medication since your symptoms can worsen.

Potential side effects of corticosteroids

Over a long period corticosteroids may cause such side effects as:

• weight gain
• thinning of the bones (osteoporosis)
• depression and mood swings
• increased risk of infection
• cataracts
• glaucoma
• worsening of diabetes or new diabetes in someone who has never had it
• thinning of the skin and easy bruising
• rounding of the face
• difficulty sleeping
• high blood pressure (hypertension)
• avascular necrosis an arthritis like condition that most commonly affects the hips or shoulders and can be treated with total hip replacement or total shoulder replacement

These medications affect everyone differently. You may have some of these side effects or none at all. If they do occur, most of these side effects will go away when the medication is stopped or decreased. Your doctor can give you some helpful tips about diet and exercise to help with some of the side effects. If you are placed on this medication it is because your doctor feels that your symptoms are serious enough to need this medication. Let your doctor know about any side effects that you may be experiencing.

You'll need to see your doctor regularly once you start taking medication. Tell your doctor how the medicine has affected your symptoms. Report any side effects that you may have developed such as weight gain or depression. Your doctor may use various tests like the ESR to adjust your medication. Even though you may feel well it is important to see your doctor regularly so you can be checked for any signs of a relapse. Once you have responded to corticosteroids the goal is to slowly reduce the dose to the lowest level necessary to control symptoms and prevent a relapse.

Special instructions for corticosteroids

Corticosteroids are different from many of the medications you may have taken in the past. Your body naturally produces small amounts of hormones that are forms of corticosteroids. When you take corticosteroids for more than a few days your body may stop producing some of these hormones. This is not permanent. Your body gradually will start making the hormones again as the dose of your medication is lowered.

Your body depends on corticosteroids for many daily functions. These hormones are important during times of stress. Your body does not care whether it receives corticosteroids from natural hormones or from medication. However if you suddenly stop taking this medication your body may not make enough hormones on its own right away. This can make you seriously ill. Also your body's need for this medicine is greatest in the morning. That is one reason why most people should take some or all of the medicine in the morning.

Some important rules about corticosteroids are as follows:

• Do not stop this medication without discussing it with your doctor.
• Do not try to decrease this medication on your own. You and your doctor will need to work together to gradually wean you off of this medicine.
• Tell your other doctors and other health professionals that you are taking this medicine. In some cases they may want to delay certain types of treatments if you are taking corticosteroids. Your medication may need to be increased for short periods of time if your body is under a lot of stress--for example if you are recovering from surgery or a serious illness. Even after you have been off corticosteroids for as long as one year you should tell your doctor that you used to take them.
• Wear medical identification to let people know that you take these medications in case a medical emergency occurs. Should you be involved in an accident or become seriously ill it is very important that this information be known. Also tell your family that you are taking corticosteroids so they can tell others in an emergency.
• If you become ill and are unable to take this medication because of vomiting let your doctor know right away. You may need to increase your usual dose for several days when you are sick.

Many of the side effects of corticosteroids are directly related to the dose given. The therapeutic goal is to always find the lowest effective dose that will avoid as many of the side effects as possible. You probably will not need large doses of corticosteroids unless your PMR is accompanied by giant cell arteritis. Serious side effects are much less common with the low doses of corticosteroids usually used in PMR but it still is wise to follow these safety rules.

What is giant cell arteritis?

Giant cell arteritis or GCA is a condition in which certain arteries (blood vessels) in the body become inflamed.

Symptoms of GCA

The symptoms of giant cell arteritis include:

• pain in the jaw muscles when eating or talking
• severe headaches
• loss of vision blurry vision or double vision--the vision loss often is described as having a curtain pulled partly over one's eye
• tenderness of the scalp or temples
• difficulty hearing
• persistent sore throat or difficulty swallowing

Loss of vision can be temporary or permanent and is the reason why higher doses of prednisone is needed. If you have PMR and start having vision problems call your doctor immediately. Also tell your doctor if you develop any of the other symptoms. It is important to diagnose and treat giant cell arteritis early to prevent blindness.

GCA incidence and risk factors

GCA can develop in some people with PMR. These two diseases often occur together. About 10-15 percent of people with PMR also have GCA.

However, GCA can occur on its own without PMR. Almost 40 percent of people with GCA also have PMR.

Causes of GCA

As with PMR the cause of GCA is unknown. Giant cell arteritis usually affects areas near the temples on the upper front sides of the head. It also involves other arteries in the head, neck, arms and occasionally will affect other arteries in the body. Inflammation causes the artery to become narrow or blocked. Inflammation can cause problems because too little blood is getting through the blood vessels.

Diagnosis of GCA

Your doctor may need to remove a small piece of an artery above and in front of your ear to determine if you have giant cell arteritis. Often the artery will be taken from the temple through a small incision. You will not need to be put to sleep to do this but you will receive medicine to numb the area. The piece of the artery then will be examined under a microscope. If you have giant cell arteritis the artery will be inflamed. A sed rate reading also can help determine the diagnosis because as in the case of PMR the sed rate is almost always higher than normal.

Treatment of GCA

Corticosteroid drugs are used to treat giant cell arteritis. Higher doses are required to treat giant cell arteritis than to treat PMR. The usual dose of prednisone for giant cell arteritis is 30-60 mg per day. Other medications are available if side effects from the corticosteroids become a problem. You can expect to stay on this treatment for many months to several years but the dose will be lowered over time once symptoms are controlled.

Credits

Some of this material may also be available in an Arthritis Foundation brochure.

Adapted from a pamphlet originally prepared for the Arthritis Foundation. This material is protected by copyright.