Paget's Disease
Medical Author: William C. Shiel Jr., MD,
FACP, FACR
What is Paget's disease?
Paget's disease is a chronic bone disorder that is due to
irregular breakdown and formation of bone tissue. Paget's disease can cause bones to expand and weaken
and may result in bone pain, arthritis, bone deformity
and fractures. It is usually localized to one bone but can involve many bones. The actual cause of Paget's disease is not known. Paget's disease is also known as osteitis deformans.
Who gets Paget's disease?
Paget's disease is rarely diagnosed in people under 40 years of age. Men and women are approximately equally affected. The
prevalence of Paget's disease ranges from 1.5%-8% depending on age and country of residence.
The prevalence of familial Paget's disease (where more than one family member has the disease)
ranges from 10%-40% in different parts of the world. Because early diagnosis and treatment is
important, after age 40, brothers, sisters and children of someone with Paget's disease may wish to
have an alkaline phosphatase blood test every two or three years. If the alkaline phosphatase level is
above normal, other tests such as a bone-specific alkaline phosphatase test, bone scan, or x-ray
can be done.
What are the symptoms of Paget's disease?
Many people do not know they have
Paget's disease because they have a mild case of the disease with no symptoms.
Sometimes, symptoms may be confused with those of arthritis or other disorders. In other
cases, the diagnosis is made only after complications have developed. Symptoms can include:
- Bone pain is the most
common symptom. Bone pain can occur in any bone affected by Paget's disease and is often worse at night. It often localizes to areas adjacent to the joints and can be accompanied by warmth to the touch.
- Headaches and hearing loss may occur when Paget's disease affects the skull.
- Pressure on nerves may occur when Paget's disease affects the skull or spine.
- Increased head size, bowing of limb, or curvature of spine may occur in advanced cases.
- Hip pain may occur when Paget's disease affects the pelvis or thighbone.
- Damage to cartilage of joints may lead to arthritis.
How is the diagnosis made of Paget's disease?
Paget's disease may be diagnosed using one or more of the following tests:
- X-rays—Pagetic bone has a characteristic appearance on x-rays.
- Alkaline phosphatase test—An elevated level of alkaline phosphatase in the blood can be suggestive of
Paget's disease but can be found in other conditions.
- Bone scan—This is useful in determining the extent and activity of the condition. If a bone scan suggests
Paget's disease, the affected bone or bones should be x-rayed to confirm the diagnosis.
What is the prognosis (outlook) with Paget's disease?
The outlook is generally good, particularly if treatment is given before major changes in the affected
bones have occurred. Paget's disease occurs most frequently in the spine, skull, pelvis,
thighs, and lower legs. In general, symptoms progress slowly, and the disease does not spread to
normal bones. Treatment can control Paget's disease and lessen symptoms, but it's not a cure.
Next: What other medical problems can be caused by Paget's disease? »
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