Overview

Overview of Paget’s Disease

Paget’s disease of bone is a chronic (long-lasting) disorder that causes bones to grow larger and become weaker than normal. The disease usually affects just one or a few bones. The bones most commonly affected by Paget’s disease include:

  • Pelvis.
  • Skull.
  • Spine.
  • Femur and tibia (leg bones).

Many people with Paget’s disease do not experience symptoms specific to the disease. However, the bone changes can cause:

  • Bone pain.
  • Misshapen bones.
  • Broken bones (fractures).
  • Inflammation and arthritis in the joints close to the affected bones.

With treatment, many people can manage their symptoms, improve pain, and control the effects of the disease.

What Happens in Paget’s Disease?

Throughout your lifetime, the body constantly removes old bone and adds new bone to the skeleton. As we age, it is normal for this process to happen at a slower pace. As the disease starts, the body loses much more bone than it makes. As Paget’s disease progresses, new bone forms at a faster rate than the rate at which old bone is removed. However, the new bone does not form correctly, leading to larger bones that are misshapen, weaker, and softer than normal bone.

Who Gets

Who Gets Paget’s Disease?

Certain factors may increase your risk for Paget’s disease:

  • Age. Paget’s disease is uncommon in people under age 40, and the chance of developing the disease increases as you age.
  • Anglo-Saxon descent. Paget’s disease is more common in certain geographical areas, including North America, Australia, New Zealand, and Europe in people of Anglo-Saxon descent. It is not common in Asia, Africa, and Scandinavia.
  • Family history. Paget’s disease commonly occurs in families.

Studies show that men may be slightly more likely than women to have the disease.

Symptoms

Symptoms of Paget’s Disease

You may not know you have Paget’s disease because many people with the disease do not have symptoms. However, fractures or misshapen bones can develop and cause pain. Sometimes, this bone pain, which is the most common symptom of the disease, is mistaken for arthritis or other disorders. Generally, symptoms develop slowly, and the disease does not spread to normal bones.

Paget’s disease can affect any bone, but usually occurs in:

  • Spine.
  • Pelvis.
  • Femur and Tibia (leg bones).
  • Skull.

In addition, some people with advanced disease may have misshapen bones and other bone changes, which may include:

  • Increase in head size.
  • Bow shape of a limb.
  • Curvature of the spine.

Other symptoms can develop, depending on the bone affected and can include:

  • Headaches and hearing loss when Paget’s disease affects the skull.
  • Tingling and numbness in arms and legs when enlarged vertebrae put pressure on the nerves in the spine.
  • Hip pain, which may occur when Paget’s disease affects the pelvis or thighbone.

Depending on the bone or bones affected by Paget’s disease, other medical problems can develop, such as:

  • Damage to joint cartilage can lead to arthritis.
  • Changes in the bones of the skull and ear can lead to hearing loss.
  • For some people who have heart disease already and severe Paget’s disease, the extra workload of pumping blood throughout the body can lead to heart failure.
  • Enlarged bones in the skull and spine can lead to pressure on the brain, spinal cord, or nerves and reduced blood flow to the brain and spinal cord.
  • When Paget’s disease affects the facial bones, the teeth may loosen. This may make chewing more difficult.
  • Rarely, people with Paget’s disease can develop bone cancer.

Causes

Causes of Paget’s Disease

Doctors do not know the cause of Paget’s disease. However, researchers suspect that environmental factors and genes play a role in the development of the disease. Scientists have identified changes in genes that may increase the chance someone could develop Paget’s disease. In addition, researchers are studying how certain viruses also may cause the disease in people who already have risk factors for Paget’s disease. Some studies suggest that, in some countries, the number of people who have Paget’s disease and the severity of the disease are decreasing.

Diagnosis

Diagnosis of Paget’s Disease

Many people with Paget’s disease are diagnosed after having tests for other conditions or to help diagnose the cause for their pain. Some common tests used to diagnose Paget’s disease include:

  • X-ray. This is the most common test that doctors use to diagnose the disease. An x-ray can evaluate the bone structure for Paget’s disease.
  • Blood test. Your doctor may order a blood test to check for the enzyme alkaline phosphatase. This test can indicate either Paget’s disease or another condition such as liver disease. If your blood contains high levels of the enzyme, it may be a sign of the disease.
  • Bone scan. A bone scan is a test that helps doctors identify which bones are affected by Paget’s disease. The test may help your doctor understand the extent of the disease. During a bone scan, a safe amount of radioactive substance is injected into a vein in your arm. The substance circulates through the bloodstream and “highlights” places in your skeleton where Paget’s disease may be present by collecting in areas where there is increased blood flow and activity of bone-forming cells characteristic of Paget’s disease.

Treatment

Treatment of Paget’s Disease

If you do not have symptoms, you may not need treatment. However, if your disease is active, you have symptoms, or you have a higher chance of developing complications, your doctor may recommend treatment. The goal of your treatment is to:

  • Slow down or stop bone breakdown and formation.
  • Lower the risk of complications.
  • Ease bone and joint pain.
  • Repair fractures.
  • Correct bone deformity

Doctors can recommend several options to help treat Paget’s disease, prevent complications of the disease and promote bone health, including:

  • Medications.
  • Surgery.
  • Exercise.
  • Diet.

Treatment can help you manage your symptoms but does not cure the disease.

Medications

The primary medications your doctor may prescribe to treat Paget’s disease include:

  • Bisphosphonates, which treat a variety of bone diseases. This type of medication works by controlling the excessive breakdown and formation of bone that happens in Paget’s disease.
  • Calcitonin, which is a hormone made by the thyroid gland. In addition, calcitonin regulates blood levels of calcium and phosphate and promotes the formation of new bone.

Surgery

Although medications can regulate bone growth and minimize complications, surgery may be necessary to correct problems from the complications of the disease, such as:

  • Fractures. Surgery may allow fractures or broken bones to heal in a better position.
  • Arthritis. A hip or knee replacement may be necessary if changes in the bones cause severe disability.
  • Misshapen bones. Cutting and realigning bone affected by Paget’s disease can reduce pain in a weight-bearing joint.

Diet

If you have Paget’s disease, you do not need a special diet. However, it is important that you keep your bones strong. Calcium and vitamin D are important nutrients to help maintain healthy bone. The chart below shows how much calcium and vitamin D you need each day. If you have had kidney stones, talk with your doctor about how much calcium and vitamin D to take.

Recommended Calcium and Vitamin D Intakes

Definitions: mg = milligrams; IU = International Units
Source: National Institutes of Health, Office of Dietary Supplements, November 2018

Life-stage group Calcium mg/day Vitamin D (IU/day)
Infants 0 to 6 months 200 400
Infants 6 to 12 months 260 400
1 to 3 years old 700 600
4 to 8 years old 1,000 600
9 to 13 years old 1,300 600
>14 to 18 years old 1,300 600
19 to 30 years old 1,000 600
31 to 50 years old 1,000 600
51- to 70-year-old males 1,000 600
51- to 70-year-old females 1,200 600
>70 years old 1,200 800
14 to 18 years old, pregnant/lactating 1,300 600
19 to 50 years old, pregnant/lactating 1,000 600

Exercise

Exercise is important because it helps:

  • Build strong bones and maintain your skeletal health.
  • Prevent weight gain.
  • Keep joints mobile.

The best exercises for healthy bones are strength-building and weight-bearing exercises, such as:

  • Walking.
  • Climbing stairs.
  • Lifting weights.

Before starting a new exercise plan, talk with your doctor to avoid stress on bones affected by Paget’s disease.

Who Treats

Who Treats Paget’s Disease?

Paget’s disease can affect many parts of the body. You may need to see more than one type of doctor, including:

  • Endocrinologists, who treat hormonal and metabolic disorders.
  • Rheumatologists, who treat joint and muscle disorders.
  • Neurologists, who treat disorders and diseases of the spine, brain, and nerves.
  • Orthopaedic surgeons, who treat bone injuries and disease.
  • Otolaryngologists, who treat ear, nose, and throat disorders.

Living With

Living With Paget’s Disease

Fortunately, current treatments can help most people with Paget’s disease lead productive lives.  When you follow your doctor’s treatment plan, you may lower the chance of developing complications or major changes in your bones. You should also:

  • Maintain a healthy weight, which is particularly important if Paget’s disease has led to arthritis of the weight-bearing joints, such as the hip or knee. Excess weight can mean more stress – and pain – for affected joints.
  • Prevent falls by:
    • Checking your home for dangers such as loose rugs and poor lighting.
    • Installing grab bars and handrails.
    • Using nonskid mats in the bathroom and tub.
    • Having regular eye exams.
    • Increasing your balance and strength by exercising every day.
  • Live a healthy lifestyle to maintain good bone health. Do not smoke, and if you do smoke, quit. If you choose to drink alcohol, do so in moderation. It is best to have no more than one drink a day for women and no more than two drinks a day for men.

Research Progress

Research Progress Related to Paget's Disease

The NIAMS supports research to help treat and possibly prevent Paget’s disease. Researchers are looking at how bone disease develops and how other areas of the body affect the development of the disease. In addition, studies are looking for genes and other factors that may contribute to Paget’s disease and are exploring possible new treatments for the disease.

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