Paget's Disease of Bone

What is Paget's disease?

Paget's disease is a long-lasting (chronic) condition that causes abnormal bone growth. Bone is constantly being replaced as bone tissue is broken down and absorbed into the body, then rebuilt with new cells. In the early stages of Paget's disease, bone tissue is broken down and absorbed much faster than normal. To keep up with the rapid breakdown of bone tissue, the body speeds up the bone rebuilding process. But this new bone is often weak and brittle, causing it to break (fracture) more easily.

Paget's disease usually affects the bones in the pelvis, spine, thigh (femur), skull, shin (tibia), and upper arm (humerus). One bone or several bones may be affected by Paget's disease.

What causes Paget's disease?

The cause of Paget's disease is not clear. Genetic factors and viruses may play a role in the development of the disease.

What are the symptoms?

Most people with Paget's disease have no symptoms. When symptoms are present, the most common are bone pain, deformed bones (bowed legs, enlarged skull or hips, or a curved backbone), and fractures. Because the bone pain may be aching and hard to describe, you may think it is part of the aging process.

Other symptoms may occur as the result of the location of Paget's disease. For example, the disease can affect the skull and cause headaches, dizziness, loss of muscle strength in the face (facial droop), or problems with vision or hearing. When the disease affects the spine, nerves can become damaged and cause leg pain, numbness, weakness, or cauda equina syndrome (an emergency condition with symptoms that include loss of feeling in the pelvic area and legs).

How is Paget’s disease diagnosed?

Most often, Paget's disease is discovered when you see a doctor or nurse for a different reason, such as hip or back pain. A bone X-ray or a blood test with above-normal levels of the enzyme alkaline phosphatase often leads to the discovery of the disease. Doctors usually diagnose Paget's disease based on your medical history, a physical exam, bone X-rays, lab tests, and possibly a bone scan.

How is it treated?

Paget's disease is treated with medicines that slow the rapid breakdown of bone tissue. You might also take medicines to relieve pain. You may need treatment to try to prevent complications such as osteoarthritis even if you do not yet have symptoms of Paget's disease. Later in the disease process, you may need surgery to replace a hip or knee joint damaged by the disease.

We don't know what causes Paget's disease. But genetic factors and viruses may play a role.

• Genetics. If your parents, brothers, or sisters have Paget's disease, you are more likely to develop the disease than if you do not have a family history of the disease.¹
• Virus. Viruses have been found in the bones of people with Paget's disease. The viruses may be associated with measles and canine distemper (found in dogs).² But there is no proof that any of these viruses cause Paget's disease.

Most people with Paget's disease have no symptoms. When there are symptoms, the most common are bone pain and bone deformities. People may have these symptoms for years before they are diagnosed as having Paget’s disease.

Paget's disease usually affects the bones in the pelvis, spine, thigh (femur), skull, shin (tibia), and upper arm (humerus). One bone (monostotic) or several bones (polyostotic) may be affected by Paget's disease.

Bone pain

Paget's disease may cause warmth, tenderness, and pain in the affected area. The bone pain tends to be worse at night and often can keep you awake. The pain does not increase when you move the affected bone and may get better with exercise. Because this pain is often aching and hard to describe, you may think it is part of the aging process. Some people with Paget's disease describe the pain as constant and deep.

The amount of bone pain may not relate to how much damage the disease is causing. You can have Paget's disease without having much bone pain, or the disease may be less severe but cause you a great deal of pain. Bones are often more painful when bone tissue is being broken down during active Paget's disease than at other times in the disease process. Pain may also be caused by a complication of Paget's disease, such as arthritis.

Bone deformities

Paget's disease may lead to bone deformities such as bowed legs, an enlarged skull or hips, or a curved backbone. Deformities are more common in long bones, such as the thighbone (femur).

A bone affected by Paget's disease can cause complications. For example, if the upper leg bows, it may change how the bones that form the hip joint and knee joint fit together and move, leading to osteoarthritis. Osteoarthritis, in turn, can cause pain and stiffness in the joints and difficulty with walking and other daily activities.

Other symptoms may occur as the result of the location of Paget's disease. For example, the disease can affect the skull and cause headaches, dizziness, loss of facial muscle strength (facial droop), or problems with vision or hearing. When Paget's disease affects the spine, it can damage nerves and cause leg pain, numbness, weakness, or cauda equina syndrome (an emergency condition with symptoms that include loss of feeling in the pelvic area and legs).

In healthy people, bone is constantly being replaced as bone tissue is broken down and absorbed into the body, then rebuilt with new cells. In the early stages of Paget's disease, bone tissue breaks down faster than it rebuilds. To make up for this breakdown process, the body speeds up the rebuilding process. But this new bone is often weak and brittle, causing it to break (fracture) more easily. Your body also tends to produce too much of this new bone, and the bone may have more blood supply than normal.

Paget's disease usually affects the bones in the pelvis, spine, thigh (femur), skull, shin (tibia), and upper arm (humerus). Paget's disease may affect just one bone (monostotic), but it usually affects more than one (polyostotic).

Paget's disease most often affects people who are older than 50 and is unusual in people younger than 40.² Early in the disease, you may not know that you have it. People often mistake symptoms such as bone pain for normal aging or osteoarthritis. Later in the disease, you may have signs such as bowed legs, enlarged skull or facial bones, stooped-forward posture, or broken bones (fractures).

Paget's disease is a lifelong illness and can be either active or inactive at different times. Paget's disease rarely spreads to additional bones after it gets started. Most people who have Paget's disease lead normal lives.

Complications of Paget's disease are rare but may include inflammation of joints (arthritis), broken bones (fractures), heart failure, and nerve problems. Nerve problems can cause a variety of symptoms, such as headache, dizziness, hearing loss, and vision problems.

Risk factors for Paget's disease include:

• Family history. If your parents, brothers, or sisters have Paget's disease, you are more likely to develop the disease than if no one in your family has it.
• Age. Paget's disease is most common in people older than 50 and is unusual in people younger than 40.²

Call your doctor immediately if you have Paget's disease and you experience signs of complications, such as:

• Sudden hearing or vision loss. As the bones in the head enlarge from Paget's disease, nerve damage can occur and can lead to permanent loss of hearing or vision.
• Weakness or numbness in your face. As the bones in the head enlarge from Paget's disease, they can damage the nerves in the face.
• Shortness of breath or severe swelling, particularly in the legs or the abdomen. This could also be a sign of heart failure.
• Weakness in both legs with loss of bladder or bowel control. This is a symptom of a specific type of nerve root pressure (compression) called cauda equina syndrome, which can result from rapid bone growth putting pressure on nerves from the spinal cord. This is a serious problem and requires immediate medical care.

Call your doctor if:

• You have symptoms of Paget's disease—such as bone pain or your skull getting larger (your hat size may increase)—and are concerned that you may have the disease.
• You have Paget's disease and have symptoms of osteoarthritis in a joint.
• You have symptoms of a pinched nerve in your back. The most common symptom of a pinched nerve is leg pain and other nerve-related (neurological) symptoms, such as tingling, weakness, or deep muscle spasms.
• You have new pain or pain that is getting worse, especially in one area of a bone or following an injury. Pain in one area may mean that a bone is broken or, rarely, may be a symptom of bone cancer.

Watchful Waiting

Watchful waiting is a wait-and-see approach. If you get better on your own (or if you don't get worse), you won't need treatment. If you get worse, you and your doctor will decide what to do next. Watchful waiting is fine for most symptoms of Paget's disease. But if you have Paget's disease and have symptoms that could be caused by a complication of this disease, do not wait to get treatment.

Who To See

Health professionals who can diagnose and treat Paget's disease include:

• General practitioner or family doctor.
• Internist.
• Endocrinologist.
• Rheumatologist.

Health professionals who can diagnose Paget's disease but usually do not treat it include:

• Orthopedist or orthopedic surgeon.
• Nurse practitioner.
• Physician assistant.

When Paget's disease is diagnosed, you may be referred to:

• An endocrinologist.
• An orthopedist, if you have a severe bone deformity, a broken bone, or severe osteoarthritis.
• An ophthalmologist, if you have vision problems.
• An otolaryngologist, if you have hearing problems.
• A rheumatologist, if you have joint problems.
• A physical therapist, if you need to start an exercise program to gain strength and endurance.

To prepare for your appointment, see the topic Making the Most of Your Appointment

Most people who have Paget's disease have no symptoms. Most often Paget's disease is discovered when you see your doctor for a different reason, such as hip pain. A bone X-ray or abnormal blood test often leads to the discovery of Paget's disease.

When your doctor or nurse suspects Paget's disease, he or she usually can diagnose it based on your medical history, physical exam, bone X-rays, lab tests, and possibly a bone scan.

Your doctor or nurse will want to be sure your symptoms are not caused by bone cancer, noncancerous tumors, fibrous tissue within the bone, or soft and deformed bones.

Lab tests Lab tests alone cannot be used to diagnose Paget's disease. Your doctor can use the results of lab tests along with results of your physical and medical exams and bone X-rays.

• Alkaline phosphatase test. The most important lab marker for Paget's disease is alkaline phosphatase, which is an enzyme produced by bone. When bone tissue grows quickly, as in Paget's disease, large amounts of alkaline phosphatase are released into the blood. Since other body organs also make this enzyme, your doctor will want to do more tests. The test for alkaline phosphatase can be used to help diagnose people who have symptoms of Paget's disease or to monitor whether the disease is active or inactive.
• Markers of bone breakdown. Both bone and cartilage contain collagen, which is released when bone tissue breaks down. Pyridinoline, deoxypyridinoline, and N-telopeptide (NTX) are fragments of collagen that may be found in urine. High levels of these substances in the urine may indicate Paget's disease. Tests for these markers may be done to monitor your response to treatment, but they are not generally used to diagnose the disease.

X-rays Several types of X-rays may be used to confirm the diagnosis of Paget's disease and help determine whether there are complications from the disease. X-ray tests may include:

• Bone X-rays. X-rays of bones affected by Paget's disease show an uneven pattern of bone growth. The affected bones often look deformed and thickened.
• Bone scan. This is the best test for diagnosing Paget's disease. You may have a bone scan of your entire body to find out which bones are affected. A bone scan is also useful for finding out whether Paget's disease is active or inactive and may show small breaks in bones that cannot be seen on a regular X-ray. However, a number of diseases can cause an abnormal bone scan.
• Magnetic resonance imaging (MRI). This test may be most useful if there are nervous system problems, such as hearing loss, vision problems, headaches, weakness or numbness of the face, or dizziness. Bone and nerves in the head affected by Paget's disease can be seen on an MRI.
• Computerized axial tomography (CAT scan). This test is most often used when bones of the skull or spine are affected by Paget's disease.

If you are diagnosed with Paget's disease, you will need regular checkups by your doctor or nurse to monitor the progress of the disease. Monitoring may include blood and urine tests that measure the amount of chemicals released from bone.

Early Detection

Most people are not screened for Paget's disease. But if you have a family history of this disease, you may want to talk with your doctor or nurse about having your blood tested (alkaline phosphatase test) when you reach age 40.

Many people do not need any treatment for Paget's disease. But the disease should be monitored for the rest of your life because of increased risk of complications such as inflammation of joints (arthritis), broken bones (fractures), and nerve problems. If your disease is active, you should see your doctor every 3 months. See your doctor or nurse every 6 to 12 months if Paget's disease is inactive.³

Doctors generally recommend treatment if you have symptoms or if you have no symptoms but are at risk of complications. You may be at risk if:

• Your lab tests suggest that your bone tissue is breaking down faster than normal.
• The disease is in long bones or bones that hold up the weight of the body (such as the thighbones or the spine). You may need treatment to prevent the affected bones from becoming brittle and breaking.
• The disease affects your skull or spine. You may need treatment for nerve problems such as dizziness, hearing loss, headaches, and numbness or tingling in the body. Paget's disease in these areas may also cause deformities (such as a bowed spine) that may make walking difficult. After bowing of bones has occurred, it cannot be reversed with medication.
• You are having bone or joint surgery. If you have Paget's disease, you need treatment with medications to slow bone growth before surgery to reduce the risk of problems such as bleeding after surgery.
• The disease requires you to stay in bed for a long time. You may need treatment to prevent levels of calcium in the blood from getting too high.

Doctors aim treatment at slowing the breakdown of bone tissue, which may help Paget's disease to become inactive. Treatment may also reduce pain and may help prevent complications.

If you have symptoms of complications from Paget's disease, such as painful joints, you may also need treatment for these conditions.

Initial treatment

For the initial treatment of Paget's disease, doctors usually prescribe a medicine called a bisphosphonate, which reduces the breakdown of bone tissue, helps control symptoms such as bone pain, and may help prevent complications such as osteoarthritis or nervous system problems. If you cannot tolerate bisphosphonate, your doctor may prescribe another medicine, called calcitonin.

Bisphosphonates nearly always make Paget's disease inactive, sometimes for years or decades. In some people, though, Paget's disease becomes active again, so you may need to take medicine off and on for the rest of your life.

If your bone pain does not go away while you are taking medicine to slow the breakdown of bone tissue, you may need acetaminophen, or aspirin, ibuprofen, or other nonsteroidal anti-inflammatory drugs (NSAIDs).

Other treatments may include the following:

• Splints or braces to support bones and joints can help prevent weakened bones from breaking. If your doctor prescribes a splint or brace, be sure it fits properly. Splints or braces that do not fit properly can cause skin irritation.
• Canes or walkers may help you walk with less risk of falling. Ask for training in how to use these devices properly. You can also use these reminders to help you use a cane or walker safely:
  • Using a cane
  • Using a walker
• Physical therapy can help strengthen muscles, increase endurance, and improve balance.
• Home health nursing may be right for you if you are taking a medicine that must be given intravenously. Home health nurses can also help make your home safe to prevent you from falling.
• Acupuncture and relaxation techniques (such as guided imagery and biofeedback techniques) may help decrease pain in some people.

You can help yourself at home by using these tips:

• Learn how to manage your pain, including using a pain diary(What is a PDF document?).
• Take precautions to avoid falling, such as keeping floors clean and dry and removing clutter and throw rugs.
• Use weight-bearing exercises, such as walking, jogging, stair climbing, dancing, or lifting weights, to strengthen bones. However, you should not place stress on bones affected by Paget's disease. Talk to your doctor or physical therapist about the best exercise program for your condition.
• Eat a balanced diet containing 1,000 to 1,500 mg of calcium and 400 IU of vitamin D each day. If you are at risk for developing kidney stones, you should talk with your doctor or nurse before adding these nutrients to your diet.
• Use a hearing aid if you have hearing loss, or use a "white noise" machine or background music if ringing in your ears is a problem.

Ongoing treatment

If you are using bisphosphonate or calcitonin medication and Paget's disease is still active, you may need to continue using it.

If the disease is now inactive, your doctor or nurse will monitor your condition with lab tests. Recommendations for these tests vary—your doctor may order the tests every 3 months to once a year. Your checkups will probably continue for life. If pain returns, or if your lab tests show active Paget's disease, you will need to start taking medicine again. Your doctor will also look for complications, such as nervous system problems.

Bisphosphonates nearly always make Paget's disease inactive, sometimes for years or decades. In some people, though, Paget's disease becomes active again. You may need to take medicine off and on for the rest of your life.

Consider or continue using splints or braces to support your weakened bones. Be sure they fit properly.

Canes or walkers may help you walk with less risk of falling. You can use these reminders on how to use a cane or walker safely:

• Using a cane
• Using a walker

You may need physical therapy to help strengthen muscles, increase endurance, and improve balance.

Acupuncture and relaxation techniques (such as guided imagery and biofeedback) may help reduce your pain.

You may have home health nursing if you are taking a medicine that must be given intravenously. Home health nurses can also help make your home safe to prevent you from falling.

You can help yourself at home by using these tips:

• Learn how to manage your pain, including using a pain diary(What is a PDF document?).
• Take precautions to avoid falling, such as keeping floors clean and dry and removing clutter and throw rugs.
• Use weight-bearing exercises, such as walking, jogging, stair climbing, dancing, or lifting weights, to strengthen bones. However, you should not place stress on bones affected by Paget's disease. Talk to your doctor or physical therapist about the best exercise program for your condition.
• Eat a balanced diet containing 1,000 to 1,500 mg of calcium and 400 IU of vitamin D each day. If you are at risk for developing kidney stones, you should talk with your doctor or nurse before adding these nutrients to your diet.
• Use a hearing aid if you have hearing loss, or use a "white noise" machine or background music if ringing in your ears is a problem.

Treatment if the condition gets worse

If Paget's disease gets worse, you may have complications such as osteoarthritis. In some cases, joint replacement surgery is necessary. Occasionally, doctors recommend surgery to relieve pressure on a nerve (such as a nerve pinched by the spine) or to correct deformed bones (such as bowed legs). Surgical choices include total joint replacement of a hip or knee and osteotomy, which may help you delay or avoid joint replacement surgery.

Paget's disease cannot be prevented. But you may prevent complications of Paget's disease, such as osteoarthritis, by taking medicine, maintaining a healthy weight, and regularly doing gentle exercise that does not cause stress to your bone.

Self-care—such as learning about the disease, avoiding undue stress on your bones, and getting regular exercise—may help you if you have Paget's disease. For example:

• Learn how to manage pain, including using a pain diary(What is a PDF document?).
• Take precautions to avoid falling, such as keeping floors clean and dry and removing clutter and throw rugs.
• Use weight-bearing exercises, such as walking, jogging, stair climbing, dancing, or lifting weights, to strengthen bones. However, you should not place stress on bones affected by Paget's disease. Talk to your doctor or physical therapist about the best exercise program for your condition.
• Eat a balanced diet containing 1,000 to 1,500 mg of calcium and 400 IU of vitamin D each day. If you are at risk for developing kidney stones, you should talk with your doctor or nurse before adding vitamin D and calcium to your diet.
• Use a hearing aid if you have hearing loss, or use a "white noise" machine or background music if ringing in your ears is a problem.

Doctors can use several types of medicine to treat Paget's disease. Although it is not possible to cure Paget's disease with them, they can make the disease inactive and may reduce complications. Medicines such as bisphosphonate and calcitonin slow the breakdown and rebuilding of bone tissue. You can use over-the-counter medicines such as acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), or other pain medicines to treat bone pain.

Medication Choices

Medications that slow the breakdown and rebuilding of bone tissue include:

• Bisphosphonates, which are usually the first medications used to treat Paget's disease.
• Calcitonin, which is usually given by injection. Your doctor probably will prescribe calcitonin if you cannot tolerate bisphosphonates or if they have not been effective in treating your symptoms.

What To Think About

You may need to take medicine for several weeks before symptoms of Paget's disease improve.

Some medicines control the disease and its symptoms only while the person is taking the medicine. Soon after the medicine is stopped, symptoms may come back.

Your doctor may recommend medicine for Paget's disease even though you have no symptoms. Medications prevent the rapid breakdown of bone tissue and may decrease the chance of complications of Paget's disease.

An oral bisphosphonate such as alendronate (Fosamax) or risedronate (Actonel) generally is used first. If the side effects are too much for you, you can try intravenous pamidronate (Aredia) or zoledronic acid (Reclast).

Joint replacements are the most common surgeries doctors use to treat joint complications from Paget's disease such as osteoarthritis of the hip or knee joints. If a joint has been damaged by this disease and is causing pain, your doctor may recommend a joint replacement (depending on the affected joint).

Osteotomy removes a wedge of bone to help reshape the remaining bone. An osteotomy for the hip or knee is another bone surgery that may help delay a joint replacement, but it is usually only a good choice for a younger person.

For more information on joint replacement surgery, see:

• Hip replacement surgery.
• Knee replacement surgery.

Occasionally, doctors may do surgery to take pressure off a nerve (such as a nerve pinched by extra bone forming in the spine) or to correct deformed bone (such as bowed legs).

If you have Paget's disease and are going to have bone or joint surgery, you may need medicine for a period of time before surgery to slow the breakdown of bone tissue and decrease the risk of bleeding during surgery.

Other treatment for Paget's disease includes:

• Splints and braces. These help support bones and joints and may be used to help prevent weakened bone from breaking. If your doctor prescribes a splint or brace, be sure it fits properly. Splints or braces that do not fit properly can cause skin irritation.
• Canes and walkers. These may help you walk with less risk of falling. Ask for training in how to use these devices properly.
  • Using a cane
  • Using a walker
• Physical therapy. This can help strengthen muscles, increase endurance, and improve balance.
• Home health nursing. You may have home health nursing if you are taking a medicine that must be given through a vein (intravenous, or IV). Home health nurses can also help make your home safe to prevent you from falling.
• Alternative therapies. Acupuncture and relaxation techniques (such as guided imagery and biofeedback) may help decrease pain in some people.

Organizations

Citations

1. Jan de Beur SM (2007). Disorders of bone and mineral metabolism: Hypocalcemia, hypercalcemia, osteomalacia, male osteoporosis, Paget disease of bone. In LR Barker et al., eds., Principles of Ambulatory Medicine, 7th ed., pp. 1437–1453. Philadelphia: Lippincott Williams and Wilkins.

2. Lane N, Leboff MS (2005). Paget's disease of bone section of Metabolic bone diseases. In ED Harris Jr et al., eds., Kelley's Textbook of Rheumatology, 7th ed., vol. 2, pp. 1487–1490. Philadelphia: Elsevier Saunders.

3. Altman RD (2005). Paget's disease of bone. In WJ Koopman, LW Moreland, eds., Arthritis and Allied Conditions: A Textbook of Rheumatology, 15th ed., vol. 2, pp. 2543–2557. Philadelphia: Lippincott Williams and Wilkins.

Other Works Consulted

• National Institutes of Health Osteoporosis and Related Bone Diseases National Resource Center (2006). Paget's disease of bone. Available online: http://www.niams.nih.gov/bone/paget.htm.