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Paget’s disease of bone

John P Walsh
Med J Aust 2004; 181 (5): 262-265.

Summary

  • Paget’s disease of bone is common, affecting up to 4% of Australians over the age of 55 years. The incidence of the disease and the severity of newly diagnosed cases appear to be falling, for unknown reasons.

  • The cause of Paget’s disease is unknown, but there is a strong genetic influence. Recently, mutations in the sequestosome 1/p62 gene have been identified as a cause of familial Paget’s disease and of some apparently sporadic cases of the disease.

  • The disease is often asymptomatic, but can cause bone pain, deformity, fracture and other complications.

  • Paget’s disease is eminently treatable. Potent bisphosphonates such as pamidronate, alendronate and risedronate relieve symptoms and may reduce the risk of complications.

  • The Pharmaceutical Benefits Scheme subsidises treatment only for patients with symptomatic disease. A strong case be made for also treating asymptomatic patients with involvement of long bones, vertebrae or base of skull, patients with significant osteolytic lesions, and perhaps all younger patients.

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  • John P Walsh

  • Department of Endocrinology and Diabetes, Sir Charles Gairdner Hospital, Perth, WA.

Correspondence: 

Acknowledgements: 

I would like to thank the Audiovisual Production Unit and the Nuclear Medicine Department at Sir Charles Gairdner Hospital for assistance with the illustrations.

Competing interests:

I have received travel assistance from Merck Sharp & Dohme to attend a meeting, and funding from Merck Sharp & Dohme and Novartis for clinical trials in Paget’s disease.

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