Paget's Disease of the Bone.

Abstract

Sir James Paget (1814–1899) began his distinguished career in the lowly position of clinical clerk to the physicians at St. Bartholomew’s Hospital, London. In due course he became assistant surgeon, and ultimately his singlemindedness and tireless application were rewarded by the achievement of eminence as a physiologist, pathologist, surgeon and teacher. Paget was an exceptional observer and writer on clinical matters; he is now chiefly remembered for his original descriptions of the two conditions that are named after him, Paget’s Disease of the Nipple and Paget’s Disease of the Bone. Paget’s descriptions of these two conditions were so perfect in the clinical aspect that practically nothing has been added to them since he first described them. 1 Paget was elected a Fellow of the Royal Society in 1851, created a baronet in 1871, and elected President of the Royal College of Surgeons in 1875. The James Paget Hospital in Great Yarmouth, the town of his birth, is named after him. Paget’s Disease of the Bone, also known as osteitis deformans, is a disease of unknown cause, characterized by localized areas of bone destruction which is followed by replacement with over-developed, light, soft, porous bone. This re-growth is associated with deformities such as thickening of portions of the skull and bending of the weight-bearing bones, but the abundant new bone is structurally abnormal and weak. Fractures of the long bones are common, and basilar invagination of the skull, internal hydrocephalus and long tract signs have also been reported. Malaise, high-output cardiac failure and cranial nerve compression, especially auditory or ocular, are also characteristic. Paget’s Disease of the Bone is most prevalent in Europe, and especially in north-west England. It is rare in patients under the age of 40, though up to 3% of the population over the age of 55 is thought to be affected (in bone disease, the incidence of Paget’s is second only to osteoporosis). Nevertheless, there is evidence that its prevalence is decreasing. 2 The patient shown here was first clinically photographed in 1974, aged 51 (Figure 1), with onset of the disease having taken place in the late 1960s. His treatment mirrors the developments in therapy that have taken place over the years. In the 1970s he received porcine, and later salmon, calcitonin, which was effective for pain relief. This drug can also assist in relieving some of the neurological complications of Paget’s Disease, such as deafness. In the early 1990s this patient received two courses of disodium etidronate, the first-generation bisphosphonate. More recently he has had further treatment with risidronate, a second-generation bisphosphonate, which clinical studies have shown to be effective in controlling Paget’s Disease, returning the alkaline phosphatase bone turnover marker in the blood to normal levels. Sarcomatous change can occur in the bones in chronic cases. The patient returned for further photography in 1985 (Figure 2) and most recently in July 2002, aged 78 (Figure 3). Overall, he exhibits the classic signs of the disease, with the skull, upper body and legs all being affected (and photographed). Notably both clavicles, and the left side in particular, demonstrate a continuing pattern of deterioration.