Paget’s disease

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

X-ray

Paget’s disease

Definition:

Paget's disease is a metabolic bone disease that involves bone destruction and regrowth which results in deformity.

Alternative Names:

Osteitis deformans

Causes, incidence, and risk factors:

The cause of Paget's disease is unknown. There are theories about early viral infection and genetic causes. The disease occurs worldwide, but is more common in Europe, Australia, and New Zealand -- where it is seen in up to 5% of the elderly population.

The disease is characterized by excessive breakdown of bone tissue, followed by abnormal bone formation. The new bone is structurally enlarged, but weakened and filled with new blood vessels.

The disease may localize to one or two areas within the skeleton, or become widespread. Frequently, bones of the pelvis, leg, spine, arm, or the collar bone are involved. The effect on the skull may enlarge head size and cause hearing loss, if the cranial nerves are damaged by the bone growth.

Symptoms:

Bone pain (may be severe and persistent)
Joint pain or joint stiffness
Headache
Bowing of the legs
Warmth of skin overlying affected bone
Fracture
Neck pain
Reduced height
Hearing loss

Note: Most patients have no symptoms.

Signs and tests:

Tests that may indicate Paget's disease include:

A bone x-ray that shows increased bone density, thickening, bowing, and overgrowth
A bone scan
Elevated serum alkaline phosphatase
Elevated markers of bone breakdown (for instance, N-telopeptide)

This disease may also alter the results of the following tests:

Treatment:

Drug therapy is aimed at suppressing bone breakdown. Currently, there are several classes of medications that inhibit bone resorption that are used in the treatment of Paget's disease. These include: bisphosphonates, calcitonin, and plicamycin.

The biphosphonates are the first-line treatment and include the following medications: etidronate (Didronel), alendronate (Fosamax), pamidronate (Aredia), tiludronate (Skelid), and risedronate (Actonel). The Calcitonin class includes intranasal (Miacalcin) and subcutaneous (Calcimar) forms. Plicamycin (Mitrhracin) may also be prescribed. Analgesics or nonsteroidal anti-inflammatory medications (NSAIDS) may be given for pain.

Localized Paget's disease requires no treatment, if there are no symptoms and no evidence of active disease. Orthopedic surgery may be required to correct a specific deformity in severe cases.

Support Groups:

Paget Foundation
120 Wall St., Suite 1602
New York, NY 10005-4001
Toll-free: 800-23-PAGET
Ph: 212-509-5335
Website: http://www.paget.org
E-mail: PagetFdn@aol.com

Expectations (prognosis):

Disease activity and symptoms can generally be controlled with current medications. Malignant changes of bone occur in less than 1% of people affected.

Complications:

Deafness
Spinal stenosis
Deformities
Bone fractures
Paraplegia
Cardiac failure

Calling your health care provider:

Call for an appointment with your health care provider if symptoms of Paget's disease develop.

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