paget's%20bone%20disease
PAGET'S BONE DISEASE
Paget's bone disease, also known as osteitis deformans, is characterized by a significant increase in bone resorption and turnover in localized parts of the skeleton causing enlargement and thickening of the bone that is disordered and architecturally unstable.
The prevalence increases with age, with men and women affected equally.
Genetic factors and/or viral infection may play a role in the etiology.
May affect one bone (monostotic) or several bones (polyostotic).
By decreasing frequency, involved bones may include pelvic bone and sacrum, spine, skull and femur, tibia, humeri and clavicles.

Diagnosis

  • Patient should be treated depending on the following:
    • Signs & symptoms of active Paget’s bone disease (eg pain at pagetic site & bone deformity)
    • Asymptomatic but w/ biochemically active Paget’s bone disease likely to cause complications in the future (eg skull base involvement that may lead to deafness)
    • Sites of pagetic lesions
    • Metabolic activity as determined by bone scan or bone turnover markers
    • Heart disease & extensive Paget’s bone disease
  • Patient w/ hypercalcemia resulting from immobilization should receive pharmacotherapy

Laboratory Tests

  • Measure biochemical markers to monitor the degree of bone formation & resorption
  • Bone turnover: Measure serum alkaline phosphatase (ALP) or bone specific alkaline phosphatase (ALP) if the previous is normal
    • Serum alkaline phosphatase (ALP) rises further if patient develops osteosarcoma
  • Bone resorption: Measure type I collagen breakdown markers (eg serum C-telopeptide or urinary N-telopeptide)
  • Serum calcium may be elevated in extensive Paget’s disease that causes immobilization
  • Urinary excretion of calcium & phosphorus may be normal or increased

Imaging

X-ray

  • Confirms the diagnosis of Paget’s bone disease
  • Findings include osteoporosis circumscripta in the skull, flame-shaped lesions in long bones, osteolytic lesions near thickened lesions, sclerotic bone, bowed limbs, fractures (ie “banana” or “chalk” transverse fractures)

Radionuclide Bone Scan

  • Most sensitive test in identifying pagetic bone lesions
  • Determines extent of bone involvement
  • May also identify possible asymptomatic bones
  • Areas of increased uptake of technetium-99m
  • “Mouse face” pattern on scan of affected vertebra

CT Scan & MRI

  • Enlarged bones w/ trabecular coarsening & increased cortical thickness may be seen in cross-sectional CT or MRI
  • Anatomy is well demonstrated in complex structures (eg spine)

Complications

  • Complications include fractures &/or fissures due to bone fragility, neurologic symptoms from nerve compression due to bone overgrowth or interference w/ blood supply, heart failure due to increased stress on the heart, & most serious complication is development of bone malignancy
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