Illness & Conditions - Health Conditions
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.
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.
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.
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 who have 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.
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).
Early in Paget's 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.
What Increases Your Risk
Things that increase your risk for Paget's disease include:
When To Call a Doctor
Call your doctor immediately if you have Paget's disease and you notice signs of complications, such as:
Call your doctor if:
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. Many experts recommend that you get treatment if you have Paget's disease in a location that could cause complications, such as in the spine, even if you are not having symptoms.
Who to see
Health professionals who can diagnose and treat Paget's disease include:
Health professionals who can diagnose Paget's disease but usually do not treat it include:
When Paget's disease is diagnosed, you may be referred to:
To prepare for your appointment, see the topic Making the Most of Your Appointment.
Exams and Tests
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.
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.
X-rays. Several types of X-rays may be used to confirm the diagnosis of Paget's disease and help find out whether there are complications from the disease. X-ray tests may include:
If you are diagnosed with Paget's disease, you will need regular checkups by your doctor or nurse to watch the progress of the disease. Monitoring may include blood and urine tests that measure the amount of chemicals released from bone.
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.
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:
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.
For the initial treatment of Paget's disease, doctors usually prescribe a medicine called a bisphosphonate, which reduces the breakdown of bone tissue and helps control symptoms such as bone pain. This medicine may also 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:
You can help yourself at home by using these tips:
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:
You may need physical therapy to help strengthen muscles, increase endurance, and improve balance.
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:
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 needed. Sometimes 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, staying at 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:
Doctors can use several types of medicine to treat Paget's disease. Although it is not possible to cure Paget's disease with medicines, 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.
Medicines that slow the breakdown and rebuilding of bone tissue include:
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. Medicines 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) typically 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:
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:
Other Places To Get Help
Last Revised: September 1, 2011
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.
Lane N (2009). Paget's disease of bone section of Metabolic bone diseases. In GS Firestein et al., eds., Kelley's Textbook of Rheumatology, 8th ed., vol. 2, pp. 1593–1595. Philadelphia: Saunders Elsevier.
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