Paget's Disease of Bone

Document Type

Article

Publication Date

1-1-1990

Description

Paget's disease of bone is thought to affect as many as 3% of people over age 40. The disease is characterized by an excessive rate of bone resorption and formation. The new bone is architecturally abnormal. Its histologic hallmarks include excessive cellularity and a disorganization in which bone lamellae run in different directions (as opposed to the concentric lamination seen in healthy bone). the number of osteoclasts is significantly increased. Their size is much greater than normal, and they have multiple nuclei. Electron microscopy reveals the presence of intranuclear inclusion bodies, suggesting a viral etiology, which, however, has not been experimentally substantiated. In fact, the cause of the disease remains unknown. In gross terms, affected bones are larger than normal. They are often deformed and fracture easily. Bone vascularity increases during active disease and declines during the subsequent quiescent phase. The expanding bones may compress nerves and interfere with their blood supply, giving rise to a number of neurologic complications. Moreover, the excessive vascularity of bones affected by Paget's disease may lead to a variety of 'steal syndromes' due to shunting of arterial blood. Many possible features, such as diminished hearing, impaired mental function, and pain, may at first not be recognized as directly related to the disease. Since the disease affects predominantly the older population, its features may instead be attributed erroneously to the aging process. The disorder's insidious onset may contribute to such errors. Physicians should be alert for Paget's disease - all the more so since effective and safe means of treatment are now available.

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