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Paget's disease
Background
Overview
Definition
Paget's disease is a chronic non-inflammatory skeletal disorder characterized by excessive and abnormal bone remodeling, resulting in deformed, enlarged, and biomechanically unstable bone.
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Pathophysiology
Paget's disease is caused by osteoclastic abnormalities due to genetic mutations (sequestosome 1 gene), with some evidence suggesting a role for chronic paramyxovirus infection.
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Epidemiology
In the US, the estimated prevalence of Paget's disease ranges from 0.9% to 3.9%.
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Disease course
In patients with Paget's disease, hyperosteoclastosis with associated fibrosis and disorganized bone deposition results in a mosaic of woven bone that is mechanically weak, and increases the risk of fracture and deformity. Platybasia, leontiasis, cranial neuropathy, radiculopathies, osteosarcomas, chondrosarcomas, and fibrosarcoma may develop as a consequence.
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Prognosis and risk of recurrence
Progression to osteosarcoma is associated with poor prognosis, even with aggressive operative management.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of Paget's disease are prepared by our editorial team based on guidelines from the Paget's Association (PA 2019) and the Endocrine Society (ES 2014).
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