Paget’s Disease Of Bone
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. Paget’s disease, otherwise known as osteitis deformans, is a chronic disorder that typically results in enlarged and deformed bones. It is named after Sir James Paget, the British surgeon who first described this disease. The excessive breakdown and formation of bone tissue that occurs with Paget’s disease can cause bone to weaken, resulting in bone pain, arthritis, deformities, and fractures.
Paget’s disease of bone causes your bones to grow larger and weaker than normal. They also might break easily. The disease can lead to other health problems, too, such as arthritis and hearing loss. You can have Paget’s disease in any bone, but it is most common in the spine, pelvis, skull and legs. The disease might affect one or several bones, but not your entire skeleton. More men than women have the disease. It is most common in older people.
Causes
Some studies have shown that up to 30% of people with Paget’s disease have other family members with it. The disease is also more prevalent in areas where much of the population is of Anglo-Saxon descent This has led some researchers to believe there may be a genetic factor in the development of Paget’s disease. A slow acting virus may also be involved, though the virus has not yet been identified.
Symptoms
If a person has a mild case with little or no symptoms, it may be several years before he or she is diagnosed. Since many people do not know they have Paget’s disease, it can be confused with arthritis, osteoporosis or other bone disorders. In many cases, the diagnosis of Paget’s disease is made only after more serious problems occur. Osteoporosis is not caused by Paget’s disease even though it is possible to have both diseases. The treatment for Paget’s disease is similar to that of osteoporosis.
Diagnosis
Diagnosis of Paget’s disease is made by physical examination, x-rays, and laboratory studies. The physical examination may show abnormalities of the skeletal shape or bone deformities. X-rays commonly show abnormalities of bone turnover, including areas of increased and areas of decreased bone deposition. Laboratory studies will invariably show an increased level of alkaline phosphatase, a byproduct of bone formation. Calcium levels within the body are usually normal.
Treatment
Treatment of the causes of Paget’s disease will usually be with a group of drugs called bisphosphonates, which are very effective. They act primarily on the osteoclasts (the bone-eroding cells) to slow down the rapid bone turnover. The choice falls between pamidronate, a drug which is injected into a vein, and two oral drugs, risedronate and tiludronate. Pamidronate is given either as a series of weekly or fortnightly injections (6 in total) or, in some centres, as a single injection which may be repeated after 6 months if necessary.
Medications that help reduce the pain associated with Paget’s include acetaminophen (e.g., Tylenol), anti-inflammatory drugs such as ibuprofen and naproxen, and a group of medications called ‘bisphosphonates.’ Bisphosphonates help the body regulate the bone building process so as to produce more normal bone growth.
