Paget's Disease
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Definition
Paget's disease is a bone condition that results in enlarged and deformed bones. It is a long-term condition. Any bone in the body can be affected. However, the most common sites are the spine, skull, pelvis, thighs, and lower legs.

Normal Bone Structure

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Causes
Paget's disease is caused by a malfunction in bone formation. Normally, bones are constantly being broken down and rebuilt. With Paget's disease, bones are broken down abnormally fast, and new bone replacement is loose and bulky, instead of strong and compact. These poorly formed bones may become weak. They also may bend over time.

The exact cause of this bone malformation is unknown. Paget's disease may be triggered early in life by a viral infection.

Risk Factors
Paget's disease is more common in people who:

  • Have a family history, especially in a parent, sibling, or child
  • Are of Northern European decent
  • Are aged 55 years or older
  • Smoke
Symptoms
Most people with Paget's disease don't have symptoms. For those with symptoms, Paget's disease may cause:

  • Chronic bone pain, especially legs, hips, or spine
  • Swelling or deformity of a limb
  • Broken bones
  • Bowing of a limb
  • Damaged cartilage in the joints
Paget's disease that involves the skull may cause:

  • Increased head size
  • Headaches
  • Facial pain
  • Hearing loss
Paget's disease doesn't spread to other bones, but the symptoms do get worse over time.

Diagnosis
Your doctor will ask about your symptoms and medical history. A physical exam will be done. Your doctor will order a blood test.

Imaging tests take pictures of internal body structures. These are done with:

Treatment
It is best to begin treatment as soon as possible. Talk with your doctor about the best treatment plan for you.

Medication
Medications for Paget's disease include:

  • Pain medications, such as acetaminophen, or non-steroidal anti-inflammatory drugs (NSAIDs)
  • Bisphosphonates
  • Calcitonin
Surgery
Surgery may be required if you have one of the following conditions:

  • Bone fracture
  • Severe degenerative arthritis
  • Bone deformity
Self-Care
Recommendations include:

  • Calcium, usually about 1,000-1,500 mg per day
  • Intake of adequate vitamin D, usually about 400 mg per day (older people may need more)
  • Regular exercise to maintain skeletal health, joint mobility, and normal body weight
  • Avoidance of excess mechanical stress on involved bones
  • A splint for an area at high risk for fracture
Prevention
There are no current guidelines to prevent the onset of Paget's disease. People with primary family members who have Paget's disease are encouraged to have a blood test every 2-3 years after age 40.




RESOURCES:
NIH Osteoporosis and Related Bone Diseases National Resource Center

The Paget Foundation

CANADIAN RESOURCES:
The Arthritis Society

Osteoporosis Canada

References:
Albagha OM, Genetic Determinants of Paget's Disease (GDPD) Consortium. Genome-wide association identifies three new susceptibility loci for Paget's disease of bone. Nat Genet . 2011;43(7):685-689.

Michou L, Brown JP. Emerging strategies and therapies for treatment of Paget's disease of bone. Drug Des Devel Ther . 2011;5:225-239.

Paget disease of bone. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what . Updated May 23, 2013. Accessed July 31, 2013.

Schneider D, Hofmann MT, et al. Diagnosis and treatment of Paget's disease of bone. Am Fam Physician . 2002;15;65(10).

What is Paget's disease of bone? NIH Osteoporosis and Related Bone Diseases National Resource Center website. Available at: http://www.niams.nih.gov/Health_Info/Bone/Pagets/pagets_disease_ff.asp . Updated May 2009. Accessed July 31, 2013.

Last Reviewed June 2013



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