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Paget's Disease

Paget's disease is a chronic disorder characterized by a breakdown and re-growth of bone tissue that typically results in enlarged and deformed bones. These enlarged bones tend to be heavy with calcification but weak overall, resulting in bone pain, arthritis, deformities, and fractures. Although another disease, osteoporosis, also produces weak bones, the two diseases are not similar otherwise.

The most common bones affected by Paget’s disease are the pelvis, leg bones, vertebrae, clavicle (collarbone), and humerus (upper arm bone). The disease is believed to be caused by a slowly progressive infection, present for many years before symptoms appear. Genetics might also play a factor in this disease since it is known to appear in more than one family member. Men and women are affected equally.

The most common symptoms of Paget’s disease include:

  • Bone pain — This is the most common symptom of Paget’s disease and can occur in any affected bone. It often affects areas near joints.
  • Headaches and hearing loss — These can occur when Paget’s disease affects the skull.
  • Pressure on nerves — This might occur when Paget's disease affects the skull or spine.
  • Hip pain — This is due to Paget's disease affecting the pelvis or thighbone.
  • Damage to cartilage of joints — This might lead to arthritis.
  • Increased head size, bowing of limb, and, rarely, curvature of spine — These are symptoms of advanced cases.

If left untreated, Paget’s disease can lead to many complications including:

  • Arthritis — Arthritis results from the long bones in the legs bowing, distorting the leg’s alignment and putting a strain on nearby joints. Bones that enlarge might also place excessive wear and tear on nearby joints.
  • Hearing — Loss of hearing in one or both ears might occur when Paget’s disease affects the skull and the bone that surrounds the inner ear. Proper treatment might slow or stop hearing loss.
  • Heart disease — In severe cases of Paget’s disease, the heart works harder to pump blood to affected bones. For people who have hardening of the arteries, this can result in heart failure if left untreated.
  • Kidney stones — Kidney stones tend to be more common in patients with Paget’s disease.
  • Nervous system — Affected bones can put pressure on the brain, spinal cord, or nerves, and reduce blood flow to the brain and spinal cord.
  • Sarcoma — In rare cases (less than 1 percent of all patients), Paget’s disease is associated with the development of a cancerous tumor of bone.
  • Teeth — Paget’s disease affecting the facial bones can result in loose teeth and difficulty chewing.
  • Vision — On rare occasions, Paget’s disease affecting the skull will affect the nerves of the eyes, causing some loss of vision.

Diagnosis

Paget's disease is rarely diagnosed in people under age 40 because many patients can have a mild case of the disease without symptoms. Also, when symptoms are present, they sometimes might be confused with those of arthritis. Because early diagnosis and treatment are important, siblings and children of someone with Paget's disease might wish to have an alkaline phosphatase blood test every two or three years after age 40. Paget's disease might be diagnosed using one or more of the following tests:

X-rays

The bones of a patient with Paget’s disease have a characteristic appearance on X-ray that the doctor will recognize.

Alkaline phosphatase blood test

An elevated level of alkaline phosphatase (an enzyme) in the blood might suggest Paget's disease.

Bone scans

This test can be used to determine the extent and activity of Paget’s disease.

Treatment and management

Although there is no cure for Paget’s disease, treatment can control disease activity and lessen the symptoms. Treatment is particularly effective if it is started before major changes in the affected bones have occurred.

Medicines

The most common types of medicine used to treat Paget’s disease are bisphosphonates. Bisphosphonates work by slowing the activity of the cells in the bones, thus slowing the progress of Paget’s disease. Some of the common brand names for bisphosphonates include Didronel, Aredia, Fosamax, Skelid, and Actonel.

Bisphosphonates are not appropriate for all patients, especially those with kidney problems. Some patients might also have allergic reactions. The doctor will determine whether or not these medicines are appropriate.

Surgery

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

  • Fractures — Surgery is sometimes performed to allow fractures to heal in better position.
  • Severe degenerative arthritis — If disability becomes severe, and medicine and physical therapy are no longer helpful, joint replacement of the hips and knees might be considered.
  • Bone deformity — The cutting and realigning of affected bones might help with painful weight-bearing joints, especially the knees.
Diet and exercise

Patients with Paget's disease should take 1000-1500 mg of calcium, be exposed to adequate sunshine, and take at least 400 units of vitamin D on a daily basis. Exercise is essential to maintaining skeletal health, avoiding weight gain, and maintaining joint mobility. A patient’s doctor and physical therapist can teach him or her how to exercise without putting any unnecessary strain on affected bones.

Paget’s disease is rarely a fatal disease and most patients can lead a healthy, active lifestyle.

Like many other conditions, education about Paget’s disease and involvement in local support groups can be the greatest tools for managing the disorder and preventing further complications. For more information on Paget’s disease, contact the following organizations:

The Paget Foundation for Paget’s Disease of Bone and Related Disorders
120 Wall Street Suite 1602
New York, NY 10005-4001
800.23 PAGET (237.2438)

National Institutes of Health
Osteoporosis and Related Bone Diseases National Resource Center
1232 22nd Street, NW
Washington DC 20037-1292
800.624.2663

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 11/4/2008...#9421

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This information is provided by Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition.

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