Paget's disease of bone

Paget's disease of bone is characterized by skeletal deformity occurring secondary to abnormal bone remodelling. Both environmental and genetic theories for the aetiology exist. The epidemiology, pathophysiology and clinical features are reviewed. Plain radiography and serum alkaline phosphatase continue to be the most widely used investigations for diagnosis and monitoring of disease activity. The mainstay of treatment is control by second generation bisphosphonates, which can now provide prolonged periods of disease remission. A minority will require surgical management due to complications arising from the disease, which can be technically challenging due to the underlying deformity, vascular nature and quality of the bone.