5 - Paget's Disease of Bone

Abstract

This chapter focuses on Paget's disease of bone. The pathological hallmark of the Paget's bone disease is excessive and disorganized activity of bone that involves both matrix and mineral. There is no idea of its cause and little knowledge of its natural history; however, the biochemical changes produced by the overactive bone justify its inclusion as a metabolic bone disease. It is the control of these abnormalities by agents that appear to act directly on bone that accounts for much of the recent interest in Paget's disease. Paget's disease is common in Britain, and the accepted overall incidence of about 3% in people older than 40 years is based on radiological or post mortem evidence. As the incidence increases rapidly with age, this 3% is the average of up to 10% in subjects in their 80s, and 1% in their 40s. Surveys refer to a selected part of the population who has been x-rayed for some medical reason, or who has come to autopsy. The incidence in the whole population is not known. The geographical distribution of Paget's disease is remarkable and inexplicable. Thus, it is almost unknown in Scandinavia and Japan, common in Australia, but rarely described in the tropics. It is said to be a disorder of the Anglo-Saxon races and their offspring.