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A Physician's Guide to The Management of Paget's Disease of Bone

Source: www.paget.org
Topic: Paget's disease of bone
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Sort Desciption: First diagnosed by Sir James Paget in 1877, Pagets disease of bone, or osteitis deformans, is a disease of the osteoclast. It is a focal disorder characterized by excessive osteoclastic bone resorption followed by excessive bone formation, resulting in bone that is architecturally unsound. ...

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What is Pagets Disease? First diagnosed by Sir James Paget in 1877, Pagets disease of bone, or osteitis deformans, is a disease of the osteoclast. It is a focal disorder characterized by excessive osteoclastic bone resorption followed by excessive bone formation, resulting in bone that is architecturally unsound. This can lead to bone pain, bone deformity and skeletal fragility. Pathology of Pagets Disease The initial abnormality in Pagets disease is a dramatic increase in the rate of bone resorption in one or more areas of the skeleton. Pagetic osteoclasts are abnormal approximately five times larger than normal containing an average of 20 nuclei per cell compared with three to four nuclei in normal adult osteoclasts. The osteoblasts, though numerous, are not abnormal. As bone resorption is coupled to bone formation, the rate of bone resorption is matched by a rapid rate of bone formation over time. The new bone is structurally disorganized, however, resulting in an overall decrease in bone strength and an increase in susceptibility to bowing and fractures. In addition, a high level of vascularity and an excess of fibrous connective tissue in the marrow mark the abnormal bone. Etiology Past studies of the etiology of Pagets disease have implicated paramyxoviruses and genetic factors in the pathogenesis of the disorder. Structures in the nuclei of osteoclasts of all patients with Pagets disease strongly resemble the nucleocapsids of viruses of the Paramyxoviridae family which includes measles virus and respiratory syncytial virus. Immunochemical staining and molecular studies have supported this impression although not all studies have been positive. Recently an animal model of Pagets disease has been developed by placing the measles virus ...

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