Definition of Paget disease

Paget disease: A chronic bone disorder that typically results in enlarged, deformed bones due to excessive breakdown and formation of bone tissue that can cause bones to weaken and may result in bone pain, arthritis, deformities or fractures. The disease is named for the great English surgeon and pathologist, Sir James Paget (1814-1899).

Paget's disease is rarely diagnosed in people under 40. Men and women are affected equally. Because Paget's disease may be familial, after age 40, brothers, sisters and children of someone with Paget's disease may wish to have an alkaline phosphatase blood test every 2 or 3 years to screen for Paget's 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. The symptoms of Paget's disease can include:

  • Bone pain -- the most common symptom. Bone pain can occur in any bone affected by Paget's disease. It often localizes to areas adjacent to the joints.
  • 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.

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.
  • Bone scan -- 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.

The outlook with Paget's disease is generally good, particularly if treatment is given before major changes have occurred in the affected bones. Paget's disease occurs most frequently in the spine, skull, pelvis, thighs, and lower legs. In general, symptoms progress slowly, and the disease does not spread to normal bones. Treatment can control Paget's disease and lessen symptoms but is not a cure.

Paget's disease may lead to other medical conditions, including:

  • Arthritis -- Long bones in the leg may bow, distorting alignment and increasing pressure on nearby joints. In addition, Pagetic bone may enlarge, causing joint surfaces to undergo excessive wear and tear. In these cases, pain may be due to a combination of Paget's disease and osteoarthritis.
  • Hearing -- Loss of hearing in one or both ears may occur when Paget's disease affects the skull and the bone that surrounds the inner ear. Treating the Paget's disease may slow or stop hearing loss. Hearing aids may also help.
  • Heart disease -- In severe Paget's disease, the heart works harder to pump blood to affected bones. This usually does not result in heart failure except in some people who also have hardening of the arteries.
  • Kidney stones -- Kidney stones are somewhat more common in patients with Paget's disease.
  • Nervous system -- Pagetic bone can cause pressure on the brain, spinal cord, or nerves, and reduced blood flow to the brain and spinal cord.
  • Sarcoma -- Rarely, Paget's disease is associated with the development of osteosarcoma, a malignant tumor of bone. When there is a sudden onset or worsening of pain, sarcoma should be considered.
  • Teeth -- When Paget's disease affects the facial bones, the teeth may become loose. Disturbance in chewing may occur.
  • Vision--Rarely, when the skull is involved, the nerves to the eye may be affected, causing some loss of vision.

Paget's disease is NOT associated specifically with osteoporosis. Although Paget's disease and osteoporosis can occur in one and the same person, they are completely different disorders. But, despite their marked differences, many treatments for Paget's disease can also be used to treat osteoporosis.

The goal of drug treatment is to control Paget's disease activity for as long a period of time as possible. Treatment options include aspirin, other anti-inflammatory medications, pain medications, and medications that slow the rate of bone turnover, such as calcitonin (Calcimar, Miacalcin) and the bisphosphonates including etidronate (Didronel), alendronate (Fosamax), pamidronate (Aredia), tiludronate (Skelid), and risedronate (Actonel).

There are generally three major complications of Paget's disease for which surgery may be recommended.

  • Fractures -- Surgery may allow fractures to heal in better position.
  • Severe degenerative arthritis -- If disability is severe and medication and physical therapy are no longer helpful, joint replacement of the hips and knees may be considered.
  • Bone deformity -- Cutting and realignment of Pagetic bone (osteotomy) may help painful weight-bearing joints, especially the knees.

Complications resulting from enlargement of the skull or spine may injure the nervous system. However, most neurologic symptoms, even those that are moderately severe, can be treated with medication and do not require neurosurgery.

In general, people with Paget's disease should receive 1000-1500 mg of calcium, adequate sunshine, and at least 400 units of vitamin D daily. This is especially important in patients being treated with bisphosphonates. Patients with a history of kidney stones should discuss calcium and vitamin D intake with their physician.

Exercise is very important in maintaining skeletal health, avoiding weight gain, and maintaining joint mobility. Since undue stress on affected bones should be avoided, patients should discuss any exercise program with their physician before beginning.

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