Paget's disease of bone

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Background

  • Characterized by areas of bone with excessive osteolytic activity and subsequent increased ostoblastic activity, resulting in disorganized bone formation
  • Results in osteolytic lesions and weakened bone
  • Eventually, affected bones may be abnormally enlarged
  • Exact etiology unknown, likely genetic component
  • Prevalence increases with age, more common in people of European descent

Clinical Features

  • Initially asymptomatic or mild pain
  • Bone pain at affected sites, often worse at night
  • Pathologic fractures
  • If skull involved, may report increased hat size, headache, decreased hearing

Differential Diagnosis

Bone tumors and their mimics

Malignant

Benign

Other

Evaluation

Paget's disease of right innominate bone.
Skull CT showing “cotton wool spots” typical of later stages of Paget’s disease.
  • Imaging to evaluate for fractures
    • Initial lesion may be destructive, radiolucent (especially in skull)
    • Involved bones may be expanded, abnormally dense
    • May have multiple fissure fractures in long bones
  • Alk phos typically very high
  • Serum calcium may be high

Management

  • Treat fractures
  • Pain control
  • Bisphosphonates (e.g. Zoledronate) are treatment of choice for patients with severe symptoms or advanced disease

Disposition

  • Discharge uncomplicated patients

See Also

External Links

References