Paget Disease

Paget disease is a condition that causes alternating cycles of bone destruction and bone reconstruction. This disease tends to slowly get worse over time.

What is going on in the body?

Paget disease alternately destroys and then reconstructs bone. Normal bone is replaced with excessive amounts of abnormal bone. The abnormal bone has less calcium and is weaker.

What are the causes and risks of the disease?

The cause of Paget disease remains unclear. Some theories about the cause of Paget disease include:

Paget disease is thought to affect about 3% of the population older than 40. The disease is twice as common in males. Paget disease is also more common in the United Kingdom, Australia, and New Zealand. It is less common in Asia, Africa, and Scandinavia. In the U.S., it is more common in the northern states and less common in the southern states.

What are the signs and symptoms of the disease?

Paget disease usually comes on gradually. In the early stages, 90% of people have no symptoms. Later, the most common symptom is a dull, mild pain in the affected bones. Often the pain is constant, present at all times of the day, and made worse by exertion. The most common locations are the skull, neck, hips, or pelvis. Because blood flow is increased in the affected bones, the affected bones may feel warm. Bones often get larger in Paget disease. When the skull is affected, there may be an increase in hat size.

Other symptoms include:

Over time, deformities such as bowlegs, a barrel chest, and other bone problems can occur.

How is the disease diagnosed?

Diagnosis of Paget disease begins with a history and physical exam. In many cases, the disease is diagnosed from routine X-rays that were taken for other reasons. Paget disease can also be seen on other special X-ray tests, such as a bone scan. Blood tests may be ordered to help in the diagnosis.

What can be done to prevent the disease?

There is no known way to prevent Paget disease.

What are the long-term effects of the disease?

Paget disease may have serious long-term effects, including:

The abnormal bone structure causes weak bones which fracture easily. Bone cancer can develop in affected areas and is usually fatal.

What are the risks to others?

Paget disease is not contagious. Some cases of Paget disease are thought to be inherited.

What are the treatments for the disease?

Often, a person with no symptoms is not treated. A person who does have symptoms is usually treated with medicines. Examples are nonsteroidal anti-inflammatory medicines such as ibuprofen. Medicines may be used to inhibit bone destruction and reduce pain in people with more severe disease. These include calcitonin and biphosphonates. Biphosphonates include risedronate, alendronate, and pamidronate.

Other treatments that are used for Paget disease include:

Long-term treatment may be needed if the disease is severe, especially if the person has nervous system problems. Adaptive equipment, such as canes and crutches, may be needed. Physical therapy may be ordered to maintain mobility and function. Surgery may be required for severely affected bones.

What are the side effects of the treatments?

All medicines can have side effects, including allergic reactions. For example, calcitonin may cause nausea and skin flushing. Herbal remedies may interfere with prescription medicines and cause bleeding problems. Surgery can cause bleeding, infection, and allergic reactions to anesthesia.

What happens after treatment for the disease?

Paget disease often requires lifelong treatment. If the symptoms go away, treatment may be stopped for a while.

How is the disease monitored?

A person with Paget disease will have regular visits with the healthcare provider. Blood and urine tests may also be performed. Any new or worsening symptoms should be reported to the provider.

Author:Jeffrey La Flamme, DC
Date Written:
Editor:Crist, Gayle P., MS, BA
Edit Date:09/05/01
Reviewer:Adam Brochert, MD
Date Reviewed:09/05/01

Sources