Paget's Disease of Bone

Medically Reviewed By William C. Lloyd III, MD, FACS
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What is Paget’s disease of bone?

Paget’s disease of bone is a chronic bone disease. Normally, bone continually undergoes a process called remodeling. Osteoclast cells constantly break down old bone and osteoblast cells are always reforming new bone. Bone remodeling helps the body maintain calcium levels in the blood. In Paget’s disease of bone, this process is abnormal. There is excessive breakdown and rebuilding happens too rapidly. The result is bone that is more fragile, softer and weaker than normal. Bones can also become misshapen and overly large.

The disease gets its name from Sir James Paget, the doctor who first described it in the 19th century. Despite knowing about the disease for more than a century, doctors still don’t have a clear understanding of exactly what causes it.

Paget’s disease of bone is uncommon. It only affects about 1% of Americans, typically older adults of northern European descent. The risk of developing it is higher if you have a family member with the disease. Men also have a higher risk than women.

In most cases, people with the disease don’t have any noticeable symptoms. When Paget’s disease of bone symptoms develop, the most common one is bone pain. The disease most frequently affects the pelvis, spine in the lower back, hips, legs, arms and skull. If these bones bend, deform or break, other symptoms can develop, such as tingling, numbness, joint pain and swelling, and hearing loss.

Treatment of Paget’s disease of the bone primarily involves medicines called bisphosphonates. Doctors commonly use this class of drugs to treat osteoporosis. They slow the breakdown of bone and relieve bone pain. Supplemental calcium and vitamin D are also prescribed to preserve healthy calcium levels. Surgery can be useful when complications develop, such as pinched nerves or misaligned bones.

Paget’s disease progresses slowly, so it isn’t a condition that requires emergency treatment. But it can cause serious problems, such heart failure, vision loss, and bone cancer. See your doctor promptly if you have new or persistent bone or joint symptoms. Regular medical care can help your doctor find potential problems when no symptoms are present.

What are the symptoms of Paget’s disease of bone?

Most people who have Paget’s disease of bone don’t realize it because they have no symptoms. They may get a Paget’s disease of bone diagnosis when they have an X-ray or other imaging exam for another reason. If symptoms develop, they can vary depending where the affected bones are in the body. The disease usually involves one bone or a few bones, but it can be widespread in some cases. Symptoms typically start slowly as the disease progresses.

Common symptoms of Paget’s disease of bone

The most common symptom of Paget’s disease of bone is bone pain. Other symptoms include:

  • Bone fractures
  • Enlarged head, one’s hat no longer fits
  • Spinal curvature, or limb disfigurement, such as bowed legs, which can lead to changes in walking gait and disability
  • Headaches, hearing loss, or vision loss
  • Loose teeth or difficulty chewing

Seeing your doctor on a regular basis can help identify signs and symptoms of bone disease early. If you notice new bone or joint symptoms, contact your doctor. Early diagnosis and treatment can help prevent complications and relieve symptoms if you have them.

What causes Paget’s disease of bone?

Paget’s disease of bone is the result of an imbalance in the bone remodeling process. Bones are not static; rather, bone tissue is continuously lost and rebuilt. This process helps the body maintain the right level of calcium in the blood. In Paget’s disease of bone, the body breaks down too much old bone and rebuilds bone too fast. The resulting new bone is abnormal, making it fragile, soft and prone to bending and breaking. This can cause the bones to become deformed and larger than normal. Unfortunately, experts do not know why it occurs, but it appears to be partially hereditary.

What are the risk factors for Paget’s disease of bone?

While the exact cause is unclear, there are known risk factors that make someone more likely to develop Paget’s disease of bone.

Risk factors include:

  • Age: It is more common in people over age 40 and is rare in people below this age. It affects about 3% of people older than 60.
  • Ancestry: It is more common in people of northern European descent, such as England and Scotland, and countries settled by these immigrants, such as Australia, New Zealand, and the United States. It is rare in Asia, Africa and Scandinavia.
  • Family history: In up to 40% of cases, there is another family member who has the disease.
  • Sex: It is more common in men compared to women.

Reducing your risk of Paget’s disease of bone

Reducing your risk of diseases and conditions relies on changing risk factors that are under your control. Unfortunately, none of the known risk factors for Paget’s disease of bone are modifiable. Because genetics appears to play a role, it’s important to know your family medical history. If a relative has had the disease, talk with your doctor about your risk. Ask how you can identify early signs of a potential problem.

How is Paget’s disease of bone treated?

Treatment may or may not be necessary if you don’t have symptoms. Your doctor can order a blood test to see if the disease is active or not. The test measures the level of an enzyme, alkaline phosphatase, in your blood. If the level is high, your doctor may recommend treating it, even if you don’t have symptoms.

Bisphosphonate drugs are the mainstay of Paget’s disease of bone treatment. This includes:

  • Pamidronate (Aredia)
  • Tiludronate (Skelid)
  • Zoledronic acid (Reclast, Zometa)

These drugs work by slowing the work of osteoclasts—the cells that break down old bone. This reduces bone loss and helps bones retain calcium and other minerals. Bones become stronger as a result. In the process, bisphosphonates can reduce bone pain.

Your doctor may also recommend physical therapy, exercise, and over-the-counter pain relievers. These strategies won’t stop the disease process, but they can help improve symptoms and maintain bone strength.

In some cases, surgery may be necessary. Surgery can help treat pinched nerves, poorly healed fractures, and misaligned bones. Joint replacement surgery may also be an option if arthritis becomes severe.

What are the potential complications of Paget’s disease of bone?

Paget’s disease of bone is a slowly progressive disease. Doctors can successfully manage most cases of the disease. However, complications are possible including:

  • Arthritis due to damage and degeneration of cartilage
  • Bone cancer, or osteosarcoma, which develops in about 1% of people with Paget’s disease of bone, usually in those over 70 years of age
  • Heart failure, which can occur if the disease is widespread as the heart works too hard to pump blood to affected areas
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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2020 Dec 1
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