Paget’s disease of the bone is a chronic ailment affecting the skeleton. Sir James Paget described Paget's disease of bone in 1877 and the disease is named after him.
Who does Paget’s disease of the bone affect?
This is a rare disease affecting 1% of the population in the United States. It commonly affects the elderly and nearly 3 to 4% of the population over age 50 are affected.
The disease commonly affects men more than women (3 men for every 2 women affected). (1-7)
What regions are affected by Paget’s disease of the bone?
Paget’s disease affects almost any bone in the skeleton but is commonest in the spine or vertebrae, pelvis or hip bones, long bones of the arms and legs and the skull.
The disease may be restricted to one bone or may affect more than one. It can affect the entire bone or a part of it.
Inheritance of Paget’s disease of the bone
The condition runs in the families and can be inherited. It can be present in as many as 25 percent to 40 percent of the direct descendants of someone with the disease.
The disease appears frequently among those of Anglo-Saxon descent and those who live in certain geographic areas, such as:
Paget’s disease is uncommon in:
It is very rare among other ethnic groups, such as Asians and Africans.
Causes of Paget’s disease of the bone
There is a marked variation in the number of cases of Paget’s disease in different regions of Britain. For example, the condition is less common in the south of the country, (estimated 1 in 100 people over the age of 55 is affected), and it is more common in the north, (county of Lancashire where 1 in 50 people over 55 may be affected).
This fact suggests that both environmental factors as well as genes may play a role in the development of Paget's disease. The exact cause is unclear.
Symptoms of Paget’s disease of the bone
The condition leads to enlarged and deformed bones. There is pathological breakdown of the bone cells. This is followed by excessive bone formation and repair that makes the bone dense but brittle.
Normally, as people age, their bones rebuild at a slower rate. For those with Paget's disease, however, this process of rebuilding and repair of bones takes place at a faster rate.
The most common symptom is bone pain. There may be recurrent fractures, joint pain or arthritis, bowing or bending of the legs, loss of hearing if the skull is affected, pinching of vital nerves leading to pain and other symptoms. Symptoms get worse slowly, and the disease does not spread to other bones.
Diagnosis of Paget’s disease of the bone
Paget’s disease is diagnosed in those over 40. Diagnosis is based on X ray features of the affected bones. Blood tests show a rise in serum alkaline phosphatase that reflects new bone formation.
Urine test results also indicate rapid bone formation. A bone scan usually shows the extent of involvement of the disease.
If bone cancer is suspected bone biopsy may be advised where samples of the affected bones are viewed under the microscope for signs of cancer.
Treatment of Paget’s disease of the bone
Medical therapies aim to reduce the frequency of pain, fractures and arthritis. Effective and safe treatment methods can help most people with Paget's disease.
Prevention of Paget’s disease of the bone
There are no known ways to prevent Paget's disease. A healthy diet rich in calcium and Vitamin D and regular physical exercise is important in maintenance of normal bone health and joint mobility.
Reviewed by April Cashin-Garbutt, BA Hons (Cantab) Further Reading