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Paget disease

PDB, Osteitis deformans

Overview

Paget disease of bone is a disorder that involves abnormal bone destruction and regrowth, which results in deformity. This condition can affect any of the bones in the body; but most people have it in their spine, pelvis, skull, or leg bones. The disease may affect only one bone or several bones; but it does not affect the entire skeleton. Bones with Paget disease may break more easily, and the disease can lead to other health problems. The cause of Paget disease is unknown, although it may be associated with faulty genes or viral infections early in life.

Symptoms - Paget disease

Many people do not know they have Paget's disease because they have a mild case of the disease with no symptoms. Sometimes, symptoms may be confused with those of arthritis or other disorders. In other cases, the diagnosis is made only after complications have developed. Symptoms can include:

  • Bone pain is the most common symptom. Bone pain can occur in any bone affected by Paget's disease and is often worse at night. It often localizes to areas adjacent to the joints and can be accompanied by warmth to the touch.
  • Headaches and hearing loss may occur when Paget's disease affects the skull.
  • Pressure on nerves may occur when Paget's disease affects the skull or spine.
  • Increased head size, bowing of limb, or curvature of spine may occur in advanced cases.
  • Hip pain may occur when Paget's disease affects the pelvis or thighbone.
  • Damage to cartilage of joints may lead to arthritis.

Causes - Paget disease

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.

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

  • Age. People older than 40 are most likely to develop Paget's disease of bone.
  • 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 close relative who has Paget's disease of bone, you're more likely to develop the condition.

 

Prevention - Paget disease

Medicine can help reduce the breakdown of bone tissue, control symptoms such as bone pain, and prevent other problems such as arthritis to improve quality of live. Currently there is no way to prevent the disease to occur.

Diagnosis - Paget disease

Paget's disease may be diagnosed using one or more of the following tests:

  • X-rays -Pagetic bone has a characteristic appearance on x-rays.
  • Alkaline phosphatase test - An elevated level of alkaline phosphatase in the blood can be suggestive of Paget's disease but can be found in other conditions.
  • Bone scan - This is useful in determining the extent and activity of the condition. If a bone scan suggests Paget's disease, the affected bone or bones should be x-rayed to confirm the diagnosis.

Prognosis - Paget disease

The outlook is generally good, particularly if treatment is given before major changes in the affected bones have occurred.In most cases, Paget's disease of bone progresses slowly. The disease can be managed effectively in nearly all people. Possible complications include:

  • Fractures and deformities. Affected bones break more easily. Extra blood vessels in these deformed bones cause them to bleed more during repair surgeries. Leg bones can bow, which can affect your ability to walk.
  • Osteoarthritis. Misshapen bones can increase the amount of stress on nearby joints, which can cause osteoarthritis.
  • Heart failure. Extensive Paget's disease of bone may force your heart to work harder to pump blood to the affected areas of your body. In people with pre-existing heart disease, this increased workload can lead to heart failure.
  • Bone cancer. Bone cancer occurs in less than 1 percent of people with Paget's disease of bone.

 

Treatment - Paget disease

The goal of treatment is to control Paget's disease activity for as long a period of time as possible.
Osteoporosis drugs (bisphosphonates) are the most common treatment for Paget's disease of bone. Some bisphosphonates are taken by mouth, while others are given by injection. Oral bisphosphonates are generally well-tolerated, but may irritate your gastrointestinal tract. Examples include:

  • Alendronate (Fosamax)
  • Ibandronate (Boniva)
  • Pamidronate (Aredia)
  • Risedronate (Actonel)
  • Zoledronic acid (Zometa, Reclast)

Rarely, bisphosphonate therapy has been linked to severe muscle, joint or bone pain, which might not resolve when the medication is discontinued. Bisphosphonates also can increase the risk of a rare condition in which a section of jawbone dies and deteriorates (osteonecrosis of the jawbone), usually associated with dental disease or a tooth extraction.

If you can't tolerate bisphosphonates, your doctor might prescribe calcitonin (Miacalcin), a naturally occurring hormone involved in calcium regulation and bone metabolism. Calcitonin is a drug that you administer to yourself by injection or nasal spray. Side effects may include nausea, facial flushing and irritation at the injection site.

In rare cases, surgery might be required to:

  • Help fractures heal
  • Replace joints damaged by severe arthritis
  • Realign deformed bones
  • Reduce pressure on nerves

Paget's disease of bone often causes the body to produce too many blood vessels in the affected bones, increasing the risk of serious blood loss during an operation. If you're scheduled for surgery that involves bones affected by Paget's disease, your doctor might prescribe medications to reduce the activity of the disease, which tends to reduce blood loss during surgery.

 

Resources - Paget disease

Refer to Research Publications.

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