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Paget's Disease of Bone
Introduction
Definition
Paget's disease of bone (osteitis deformans) is a focal disorder of bone resorption followed by excessive bone formation. The primary disturbance is an exaggeration of osteoclastic bone resorption, initially producing a localized bone loss. The disorder is usually not recognized until the subsequent bone formation response is so pronounced that enlarged and deformed bones result. This excessive resorption and formation culminates at the tissue level in an abnormal mosaic pattern of lamellar bone associated with extensive vascularity and increased fibrous tissue deposition in adjacent marrow spaces.
- Complications involve the bones (fractures and neoplastic degeneration), joints (osteoarthritis), and nervous system.
- The short-term objective is to alleviate bone pain and the long-term objective is to minimize or prevent the progression of the disease (Delmas 1997).
Prevalence and Epidemiology
The incidence of Paget's disease is difficult to estimate, because most patients are asymptomatic. The information that is available is derived from autopsy studies and from plain radiographs obtained on hospital admissions of unselected patients. These studies found Paget's disease in 3 to 3.7 percent of patients over the age of 40 years, which makes it the 2nd most common disorder of bone, outstripped only by osteoporosis (Polednak 1987). The disease is seldom encountered before the age of 40, but its incidence approximately doubles each decade from the age of 50 onwards (Kanis 1991). It affects males more than females in a proportion of 3:2.
The exact prevalence varies greatly between countries, and even within countries. Clinical observations indicate that the disease is most common in Europe, North America, Australia and New Zealand. The highest prevalence can be found in England (4.6%) and France (2.4%), with other Western European countries reporting a slightly lower prevalence (Barker 1984). It is conspicuously rare in Scandinavia, India, Japan, China, the Arab Middle East and Black Africa. In the USA, a higher prevalence is seen in the northern part of the country than in the south (Guyer 1980).
Scope
The purpose of this chapter is to provide an overview of the pathophysiology, etiology, diagnosis, clinical manifestations and management of Paget's disease of bone.
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