Paget's disease of the bone is a rare and debilitating disease. It is a sporadic disease usually involving multiple bones though occasionally isolated sites may occur. The most common sites include the lumbosacral spine, pelvis, and skull. This is an important diagnosis because of the potential for malignant tumors to develop in the diseased bone.
Patients may present with a variety of signs and symptoms depending upon which bones are affected. Arthritis, loss of hearing in one or both ears, heart disease (high output cardiac failure secondary to the heart working harder to pump blood to affected bones, kidney stones, nervous system abnormalities secondary to Pagetic bone causing pressure on the brain, spinal cord, or nerves, and reduced blood flow to the brain and spinal cord.
The teeth and vision may also be affected when bones of the skull are involved. The survey of Paget's disease in 10 Belgium towns suggests a prevalence of 2.5% among men and 1.6% among women aged 55 years and over. Age-adjusted prevalence rates declined steeply between 1974 and 1994. These declines suggest an environmental contribution to the etiology of this disorder that requires further investigation.