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Paget's Disease (cont.)

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What are the types of Paget's disease?

There are two types of Paget's disease of bone. When a single site of bone is affected by Paget's disease, it is referred to as the monostotic type. When multiple sites of bone are affected by Paget's disease, it is referred to as the polyostotic type.

What causes Paget's disease?

It is not known what causes Paget's disease. Paget's disease is felt to be a disease of a particular cell that is responsible for bone resorption, the osteoclast.

What are risk factors for Paget's disease?

The onset of Paget's disease is typically after 55 years of age. Recently, certain genes have been associated with Paget's disease, including the Sequestrosome 1 gene on chromosome 5. Virus infection may be necessary to trigger Paget's disease in people who have inherited the genetic tendency to develop the condition by having these genes. Paget's disease is inherited in approximately one-third of patients. Therefore, it is not uncommon for a patient with the condition to have a family history of the disease.

Who discovered Paget's disease?

Paget's disease is named after the English surgeon Sir James Paget, who described the condition in 1877. Paget also discovered the worm that causes trichinosis and described what is called Paget's disease of the breast. Together with Rudolph Virchow in Germany, Paget was one of the founders of pathology.

What are Paget's disease symptoms and signs?

Paget's disease causes no symptoms in a majority of those with the condition and is often incidentally noted when X-ray tests are obtained for other reasons. However, Paget's disease can cause bone pain, deformity, fracture, and arthritis. The bone pain of Paget's disease is located in the affected area of bone. The most common bones affected by Paget's disease include the spine, the thigh bone (femur), the pelvis, the skull, the collarbone (clavicle), and the upper arm bone (humerus). Fractures can occur because affected bone is not as strong as normal bone. Arthritis occurs because of the deformity of affected bone adjacent to the joint and because of bowing of affected long bones. The type of arthritis that results is osteoarthritis.

The symptoms of Paget's disease depend on the bones affected and the severity of the disease. Enlarged bones can pinch adjacent nerves, causing tingling and numbness. Bowing of the legs can occur. Hip or knee involvement can lead to arthritis, limping, as well as pain and stiffness of the hip or knee. Headache, loss of vision, and hearing loss can occur when bones of the skull are affected. With very widespread Paget's disease, it is possible to develop congestive heart failure due to an increased workload on the heart. Sometimes the skin overlying the involved bone is warmer than usual. This is because of the increased blood supply to pagetic bone.

Medically Reviewed by a Doctor on 2/28/2017


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