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Paget's disease Alternative Names
osteitis deformans
Definition
Paget's 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?
As Paget's 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 because its structure is different.
What are the signs and symptoms of the disease?
Paget's 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's disease. When the skull is affected, there may be an increase in hat size.
Other symptoms include hearing loss, unexplained bone fractures, headaches, dizziness, and ringing in the ears. Over time, deformities such as bow legs, a barrel chest, and other bone problems can occur.
What are the causes and risks of the disease?
The cause of Paget's disease remains unclear. Some theories about the cause of Paget's disease include: - an inflammatory condition, or an immune response to injury
- a hormone disorder, in which the body produces abnormal amounts of hormones that regulate bone maintenance
- an autoimmune disorder, in which a person's immune system attacks his or her own body
- a metabolic disorder, or abnormalities in physical and chemical processes within the body
- a slow viral infection
Paget's disease is thought to affect about 3% of the population older than 40. The disease is twice as common in males. Paget's disease is also more common in the United Kingdom, Australia, and New Zealand. It is less common in Asia, Africa, and Scandinavia. In the US, it is more common in the northern states and less common in the southern states.
X-ray evidence indicates that Paget's disease was present in Anglo-Saxon skeletons 1000 years ago. This suggests a natural environmental link. An analysis of Beethoven's skeleton suggests that Paget's disease may have contributed to his hearing loss.
What can be done to prevent the disease?
There is no known way to prevent Paget's disease.
How is the disease diagnosed?
In many cases, the disease is diagnosed from routine x-rays that were taken for other reasons. The replacement of normal bone by the abnormal bone can be seen on x-rays. The way the bones look on x-rays has been called a "mosaic" structure. The skull or bones in the spine may look larger and thicker. Pelvic changes and bowing or bending of the long bones, mainly the thighbone, are also common. Bone destruction and other lesions are often visible. Paget's disease can also be seen on other special x-ray tests, such as a bone scan.
Other findings include high levels of an amino acid in the urine and high levels of a bone enzyme in the blood.
What are the long-term effects of the disease?
In severe cases, Paget's disease can cause death. Complications include kidney stones, congestive heart failure, anaemia or low red blood cell counts, paralysis, deafness, and blindness. 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?
Generally, there are no risks to others. Some cases of Paget's 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 medications, such as non-steroidal anti-inflammatory medications like ibuprofen. Medications used for more severe disease include calcitonin and biphosphonates. These help inhibit bone destruction and reduce pain. Long-term treatment is needed if the disease is severe, especially if the person has nervous system problems. Surgery may be required for severely affected bones.
Other treatments include calcium, magnesium, and glucosamine to support bone growth. Herbal remedies include primrose oil, alfalfa, valerian root, and capsicum to reduce pain and inflammation. A person with Paget's disease is advised to eat foods that are rich in calcium. These include brewer's yeast, goat's milk, leafy greens, salmon, tofu, and yoghurt. It may also help to avoid the nightshade vegetables. These vegetables, which affect the metabolism of calcium, include tomatoes, potatoes, eggplant, and capsicum.
What are the side effects of the treatments?
All medications can have side effects, including allergic reactions. For example, calcitonin may cause nausea and skin flushing. Herbal remedies may interfere with prescription medications. A person should let the doctor know about any herbal remedies used. All surgery carries a risk of bleeding, infection, and reactions to anaesthesia.
What happens after treatment for the disease?
In many people, treatment in some form continues for life. If the symptoms go away, treatment may be stopped for a while.
How is the disease monitored?
Symptoms are monitored and regular physical examinations are done. Blood and urine tests may also be performed.
Author: Jeffrey La Flamme, DC
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request |
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