Table of contents
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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Diagnostic investigations
Laboratory evaluation: as per PA 2019 guidelines, obtain serum total ALP (in conjunction with liver biochemical tests) as part of the initial laboratory evaluation for the presence of metabolically active Paget's disease.
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Plain radiographs
Radionuclide bone scan
MRI and CT
Medical management
Bisphosphonates: as per PA 2019 guidelines, initiate bisphosphonate therapy for the treatment of bone pain associated with Paget's disease. Use zoledronic acid as the bisphosphonate most likely to give a favorable pain response.
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Calcitonin
Denosumab
Treatment targets
Management of spinal cord dysfunction
Surgical interventions
Management of bone fractures
As per PA 2019 guidelines:
Perform surgery for fixation of fractures through affected bone in patients with Paget's disease, recognizing that the clinical outcome in femoral neck and subtrochanteric fractures is poor.
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Insufficient evidence to recommend one type of surgical treatment over another.
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Management of osteoarthritis
Management of spinal stenosis
Specific circumstances
Patients with hearing loss: as per PA 2019 guidelines, insufficient evidence to recommend bisphosphonate therapy to prevent progression of hearing loss in patients with Paget's disease.
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Patients with osteoarthritis
Patients undergoing orthopedic or spinal surgery
Patients with osteosarcoma
Patients with congestive HF
Patient education
Counseling on bisphosphonates: as per PA 2019 guidelines, inform patients undergoing treatment with bisphosphonates for Paget's disease that this class of medication has a favorable adverse event profile. Advise patients that a transient flu-like illness occurs commonly with intravenous zoledronic acid.
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