Paget's disease of bone
| Paget's disease of bone | |
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| Synonyms | osteitis deformans, Paget's disease |
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| "This 92 year-old male patient presented for assessment of sudden inability to move half his body. An incidental finding was marked thickening of the calvarium. The diploic space is widened and there are ill-defined sclerotic and lucent areas throughout. The cortex is thickened and irregular. The findings probably correspond to the “cotton wool spots” seen on plain films in the later stages of Paget’s disease." | |
| Pronunciation |
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| Classification and external resources | |
| Specialty | rheumatology |
| ICD-10 | M88 |
| ICD-9-CM | 731.0 |
| OMIM | 602080 |
| DiseasesDB | 9479 |
| MedlinePlus | 000414 |
| eMedicine | med/2998 radio/514 pmr/98 |
| Patient UK | Paget's disease of bone |
| MeSH | D010001 |
Paget's disease of bone (also termed osteitis deformans or , just Paget's disease) is caused by the excessive breakdown and formation of bone, followed by disorganized bone remodeling. This causes affected bone to weaken, resulting in pain, misshapen bones, fractures and arthritis in the joints near the affected bones. Rarely, it can develop into a primary bone cancer known as Paget's sarcoma. Often Paget's disease is localized to only a few bones in the body. The pelvis, femur, and lower lumbar vertebrae are the most commonly affected bones. Paget's disease typically is localized, affecting just one or a few bones, as opposed to osteoporosis, for example, which usually affects all the bones in the body.
A later phase of the disease is characterized by the replacement of normal bone marrow with highly vascular fibrous tissue. Because early diagnosis and treatment is important, after age 40, siblings and children of someone with Paget's disease should 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 performed.
Decisions about treating Paget's disease may be complicated as the disease often affects people differently. In addition to this, it is sometimes difficult to predict whether a person with Paget's disease, who shows no signs or symptoms of the disorder, will develop symptoms or complications (such as a bone fracture) in the future. Although there is no cure for Paget's disease, medications (bisphosphonates and calcitonin) can help control the disorder and lessen pain and other symptoms. Medications are often successful in controlling the disorder, especially when started before complications begin.
Paget's disease affects from 1.5 to 8.0 percent of the population, depending on age and country of residence. Paget's disease is rare in people less than 55 years of age. Men are more commonly affected than women (3:2). The disease is named after Sir James Paget.
Many people with Paget's disease do not know they have the disease, because they have a mild case with no symptoms. In addition, when symptoms do occur, they may be confused with those of arthritis or other disorders. In other cases, the diagnosis is made only after complications have developed. About 35 percent of patients with Paget's have symptoms related to the disease when they are first diagnosed.
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Wikipedia