Health Encyclopedia

Paget’s disease

Definition

Paget's disease is a disorder that involves abnormal 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, although it might have to do with genes or a viral infection early in life.

The disease occurs worldwide, but is more common in Europe, Australia, and New Zealand.

In people with Paget's, there is an abnormal breakdown of bone tissue, followed by abnormal bone formation. The new bone is bigger, but weakened and filled with new blood vessels.

The disease may only be in one or two areas of the skeleton, or throughout the body. It often involves bones of the:

Arm
Collar
Leg
Pelvis
Spine

Symptoms

Bone pain (may be severe and persistent)
Bowing of the legs and other visible deformities
Enlarged head
Fracture
Headache
Hearing loss
Joint pain or stiffness
Neck pain
Reduced height
Skull deformities
Warmth of skin over the affected bone

Note: Most patients have no symptoms.

Signs and tests

Tests that may indicate Paget's disease include:

Bone scan
Bone x-ray
Elevated markers of bone breakdown (for instance, N-telopeptide)
Elevated serum alkaline phosphatase

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

Treatment

Not all patients need treatment. For example, patients who have abnormal blood tests only may not need treatment.

People with Paget's disease who are commonly treated include:

Patients with deformities
Patients with no symptoms when certain bones (such as weight-bearing bones) are involved, especially if the bony changes are progressing quickly, to reduce the risk of fractures
Patients with symptoms

Drug therapy helps prevent further bone breakdown. Currently, there are several classes of medications used in the treatment of Paget's disease. These include:

Bisphosphonates -- These drugs are the first-line treatment, and they help increase bone density. Types of bisphosphonates include:
- Alendronate (Fosamax)
- Etidronate (Didronel)
- Pamidronate (Aredia)
- Risedronate (Actonel)
- Tiludronate (Skelid)
- Zoledronic acid (Zometa)
Calcitonin -- This hormone is involved in bone metabolism. Types include:
- Intranasal (Miacalcin)
- Subcutaneous (Calcimar)
Plicamycin (Mithracin)

Analgesics or nonsteroidal anti-inflammatory medications (NSAIDS) may also be given for pain.

Localized Paget's disease needs 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. A small percentage of patients may develop a cancer of the bone called osteosarcoma. Some patients will need joint replacement surgery.

Complications

Bone fractures
Deafness
Deformities
Heart failure
Paraplegia
Spinal stenosis

Calling your health care provider

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

References

Goldman L, Ausiello D. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007.

Review Date:12/6/2007
Reviewed By:Nancy J. Rennert, M.D., Chief of Endocrinology Diabetes, Norwalk Hospital, Associate Clinical Professor of Medicine, Yale University School of Medicine, New Haven, CT. Review provided by VeriMed Healthcare Network.

ADAM Quality A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.

Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.