Your Health - Paget's Disease

Paget's Disease

(Osteitis Deformans)

Definition

Paget's disease is a chronic condition that results in enlarged and deformed bones. Although any bone in the body may be affected, the most common sites are the spine, skull, pelvis, thighs, and lower legs. The disease does not usually spread to other normal bones.

Causes

Paget's disease is caused by a malfunction in bone formation. Normally, bones are constantly being broken down by cells called osteoclasts, and rebuilt by cells called osteoblasts. 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, and may bend over time.

Normal Bone Structure

The exact cause of this bone malformation is unknown, but it is associated with heredity. Some experts believe that Paget's may be triggered early in life by a viral infection.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Risk factors for Paget's disease include:

Family history of Paget's disease, especially in a parent, sibling, or child
Race: Anglo-Saxon, European
Age: 40 or older
In US, residence in northern states

Symptoms

In the early stages, most people have no noticeable symptoms. In later stages, symptoms may include:

Swelling or deformity of a limb
Increased head size
Bowing of a limb
Curvature of the spine
Chronic bone pain, especially legs, hips, or spine
Frequent fractures
Problems with walking or gait
Headaches, facial pain, or hearing loss when the skull bones are affected
Swollen or painful joints due to damaged joint cartilage
Numbness, tingling, pain, or weakness from pressure on nerves

Depending on the bone site, Paget's disease may result in:

Arthritis, usually osteoarthritis
Carpal tunnel syndrome
High calcium in blood
Kidney stones
Hearing problems
Dental problems
Rarely:
- Eye problems
- Congestive heart failure
- Bone cancer (sarcoma)

Diagnosis

The doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include:

Blood tests—such as those for alkaline phosphatase or calcium
X-ray —a test that uses radiation to take a picture of the bones
Bone scan —a series of pictures of bones taken after injection of a small amount of radioactive material

Treatment

Treatment may include:

Medication

Medications may include:

Pain medicine—such as acetaminophen (Tylenol) or nonsteroidal anti-inflammatory drugs
Bisphosphonates—usually by tablet or by injection
Calcitonin —administered by injection or nasal spray

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
Adequate exposure to sunshine to promote vitamin D production in the skin (but limit time in the sun to prevent sun burning, wrinkling, and aging)
Intake of adequate vitamin D , usually about 400 mg per day (more may be needed in older people)
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 is no known way to prevent the onset of Paget's disease. People with primary family members who have Paget's disease are encouraged to have a routine alkaline phosphatase blood test every two to three years after age 40.

RESOURCES:

Osteoporosis and Related Bone Diseases National Resource Center
http://www.niams.nih.gov/Health_Info/Bone/

The Paget Foundation
http://www.paget.org/

CANADIAN RESOURCES:

The Arthritis Society
http://www.arthritis.ca/

Osteoporosis Canada
http://www.osteoporosis.ca/

References:

National Institutes of Health Osteoporosis and Related Bone Diseases, National Resource Center website. Available at: http://www.niams.nih.gov/Health_Info/Bone/ . Accessed October 11, 2005.

Questions and answers about Paget's disease of bone. The Paget Foundation website. Available at: http://www.niams.nih.gov/Health_Info/Bone/ . Published 2008. Accessed July 24, 2009.

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

Last reviewed November 2009 by Robert E. Leach, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.