Pagets Disease

Paget's disease of bone interferes with your body's normal recycling process, in which new bone tissue gradually replaces old bone tissue. Over time, bones can become fragile and misshapen. The pelvis, skull, spine and legs are most commonly affected.

Causes #

• The cause of Paget’s disease of bone is unknown. Scientists suspect a combination of environmental and genetic factors contribute to the disease. Several genes appear to be linked to getting the disease.

• Some scientists believe Paget’s disease of bone is related to a viral infection in your bone cells, but this theory is controversial.

Risk factors #

Factors that can increase your risk of Paget’s disease of bone include:

• Age - People older than 50 are most likely to develop the disease.

• Sex - Men are more commonly affected than are women.

• National origin - Paget’s disease of bone is more common in England, Scotland, central Europe and Greece as well as countries settled by European immigrants. It’s uncommon in Scandinavia and Asia.

• Family history - If you have a relative who has Paget’s disease of bone, you’re more likely to develop the condition.

Symptoms #

• Most people who have Paget’s disease of bone have no symptoms. When symptoms occur, the most common complaint is bone pain.

• Because this disease causes your body to generate new bone faster than normal, the rapid remodeling produces bone that’s less organized and weaker than normal bone, which can lead to bone pain, deformities and fractures.

The disease might affect only one or two areas of your body or might be widespread. Your signs and symptoms, if any, will depend on the affected part of your body.

• Pelvis - Paget’s disease of bone in the pelvis can cause hip pain.

• Skull - An overgrowth of bone in the skull can cause hearing loss or headaches.

• Spine - If your spine is affected, nerve roots can become compressed. This can cause pain, tingling and numbness in an arm or leg.

• Leg - As the bones weaken, they may bend causing you to become bowlegged. Enlarged and misshapen bones in your legs can put extra stress on nearby joints, which may cause osteoarthritis in your knee or hip.

Treatment #

Nonsurgical Treatment #

• If you do not have pain or other symptoms, no treatment is required.

• Your doctor may recommend simply monitoring your condition with regular office visits and periodic X-rays to watch for changes in the affected bone and to ensure that complications do not develop.

• If symptoms do occur, your doctor may recommend one or more nonsurgical treatments.

• Nonsteroidal anti-inflammatory drugs (NSAIDs) - Medications such as ibuprofen, naproxen, and aspirin can help relieve mild bone pain that arises from Paget’s disease or from arthritis that may be associated with the disease.

• Assistive devices - If your pelvis or leg is affected by the disease, using a cane can help relieve pain by decreasing the forces going through the bone. Using a cane can also help prevent falls, so there is less risk of fracture in the bone — a common complication of Paget’s disease.

• Wearing a brace can help relieve pain by preventing malalignment of the affected bones.

• Bisphosphonate medications - When bone pain is more significant, medications called bisphosphonates are the treatment of choice. These drugs block osteoclasts and can be very effective in treating Paget’s disease.

• There are several types of bisphosphonates. Some are given by mouth (orally) and others are given as an injection (intravenously). Your doctor will talk with you about which type is best for you and how long you will need to take it.

• During treatment with bisphosphonates, your doctor will perform blood tests periodically to check your alkaline phosphatase level. A falling alkaline phosphatase level and improvement in bone pain are indications that treatment is working.

Diagnosis #

Laboratory tests. #

• A blood test called serum alkaline phosphatase may also be used to help confirm the diagnosis.

• In patients with Paget’s disease, alkaline phosphatase levels are usually quite elevated a reflection of the high bone turnover rate.

• Paget’s disease can also be detected with urine tests that show rapid bone turnover.

Bone scan. #

• A bone scan may be used to help determine which bones are affected. During this test, a very small amount of radioactive dye is injected into a vein.

• A special camera is then used to detect areas of the skeleton with an increased uptake of the radioactive material. These hot spots indicate areas where there is more bone turnover than normal.

• Paget’s disease almost always looks hot on a bone scan, except when the condition has been present for a long time and has burned out.

Biopsy. #

A biopsy is sometimes necessary to confirm the diagnosis of Paget’s disease or to rule out other conditions.

Surgical Treatment #

In some cases, surgery may be needed to treat the complications of Paget’s disease, including:

• Bone fractures

• Malalignment or deformity of bone

• Severe arthritis The surgical procedures used to treat fractures, malalignment, or arthritis in patients with Paget’s disease are similar to those used to treat similar conditions in people with normal bone.

These procedures may include:

Internal fixation #

This procedure can be used to treat fractures in bone affected by the disease. In internal fixation, bone fragments are first repositioned into their normal alignment, then held in place with screws, wires, pins, or metal plates attached to the outside of the bone.

Osteotomy #

An osteotomy can help relieve pain and restore alignment to weight-bearing joints that are affected by Paget’s disease, especially the knee and hip. During the procedure, your doctor will remove a wedge of bone near the damaged joint in order to shift weight onto a healthier part of the joint.

Total joint replacement #

In this procedure, parts of an arthritic or damaged joint are removed and replaced with a metal, plastic or ceramic device called a prosthesis. The prosthesis is designed to replicate the movement of a normal, healthy joint.