Paget's disease of bone is an uncommon, chronic bone disorder that occurs in only about one percent of people in the United States and slightly more often in men than in women (three to two). Individuals with Paget's disease experience rapid isolated bone repair, which causes a variety of symptoms from softer bones to enlarged bone growth, typically involving one or more bones of the pelvis, low back (spine), hips, thighs, head (skull) and arms. This can lead to problems such as bending, breaking, pinched nerves, arthritis and reduced hearing. Medical therapies have proven effective in reducing the frequency of pain, fractures and arthritis that may be caused by this condition.
Normally, as people age, their bones rebuild at a slower rate. For those with Paget's disease, however, this process of rebuilding the bones involved take place at a faster rate. As a result, the rebuilding bone has an abnormal structure. The involved bone can be soft, leading to weakness and bending of the pelvis, low back (spine), hips, thighs, head and arms. Or, the rebuilt bone can enlarge, making it more susceptible to arthritis, hearing loss, fractures and discomfort. Given that this takes place in those over the age of 40, the symptoms often are mistaken for changes associated with aging.
The cause of Paget's disease is unknown. It does appear to be, at least partially, due to hereditary factors, perhaps activated by exposure to a virus. It is rarely discovered in individuals before they reach the age of 40, and the number of people identified increases in each progressive age group. Indicative of the heredity consideration, Paget's disease occurs more commonly in European populations and their descendants. In 30% of cases, disease incidence often involves more than one member of a family.
Paget's disease often causes an arthritic condition and can be diagnosed and treated by your rheumatologist as well as medical care practitioners who focus on bone disorders.
Typically, it is the appearance of the bones on an X-ray that signals the physician to make the diagnosis. Blood tests taken most often will indicate an increase in serum alkaline phosphatase (SAP), which is reflective of the rapid new bone turnover. Urine test results also will indicate the speed at which this rebuilding is taking place. Physicians usually obtain a non-invasive bone scan to determine the extent of bone involvement. Only if cancer is suspected will it be necessary to biopsy the bone to examine it under a microscope.
Treatment for Paget’s disease can focus on providing physical assistance, including the addition of wedges in the shoe, canes as walking aids and the administration of physical therapy.
Medications that help reduce the pain associated with Paget's disease include acetaminophen (e.g., Tylenol) and anti-inflammatory drugs such as ibuprofen and naproxen. In addition, a group of medications called bisphosphonates reduce the pain and help the body regulate the bone-building process to stimulate more normal bone growth. Your physician may prescribe an oral medication:
All oral medications should be taken with a large glass of water (6-8 oz) upon arising in the morning. Patients should remain upright for the next 30 minutes and should not eat, drink or take other medications until that time has passed. Any of these treatments can be repeated if necessary. Side effects of these medicines may involve heartburn and sometimes increasing bone pain for a short period of time.
There are also injectable medications. Injectable medications that can be given for Paget's include:
Occasionally surgery is needed to help arthritis caused by the bone changes of Paget's disease and can be helpful in reducing pain and improving function. Medical treatment is not expected to correct some of the changes of the Paget's disease that have already occurred, such as hearing loss, deformity or osteoarthritis.
Paget's disease does not seriously affect quality of life, and, for most people, the outcome is good. In fact, most people with Paget's disease of bone have no complaints. Instead, it is the fast bone rebuilding that leads to complications such as bone pain, enlarged head, bowed arms or legs, arthritis (osteoarthritis), back pain, loss of hearing, broken bones (fractures), heart failure and, rarely, a form of bone cancer.
Updated December 2021 by the American College of Rheumatology Committee on Communications and Marketing.
This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.