Paget Disease is a topic covered in the 5-Minute Emergency Consult.

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Basics

Description

  • Paget disease involves resorption of normal bone and its replacement with fibrous and sclerotic tissue
  • Also known as osteitis deformans
  • Usually focal, bones most frequently involved include:
    • Pelvis (70%)
    • Femur (55%)
    • Skull (42%)
    • Tibia (32%)
    • Spine (53%, lumbar spine)
    • Flat bones
  • Usually found incidentally and generally asymptomatic
  • Occurs in ∼1–2% of patients >55 yr old
  • Incidence increases with age
  • Starts with resorptive or osteolytic phase, during which osteoclasts remove healthy bone
  • Hypervascularity begins in resorptive phase:
    • Predisposes to hematoma and fracture
  • Resorbed bone is eventually replaced by irregular, dense, disorganized trabecular bone in sclerotic or osteoplastic phase forming “mosaic pattern”
  • Malignant transformation is rare:
    • Osteosarcoma is malignancy of concern
    • Usually malignant transformation occurs in 1%
  • More common in men
  • More common in European descent
  • Less common in Asian or Scandinavian descent
  • Typically involves 1 bone (monostotic)
  • May involve a few bones (polyostotic)

Etiology

  • Unknown
  • Genetic component:
    • SQSTM1 mutation seen in many but not all cases
  • Environmental influences may also play a role
    • Presence of nucleocapsids from measles, canine distemper, paramyxovirus, or respiratory syncytial virus may implicate viral cause
    • Possible association with rural life and close contact with farm animals
  • May represent vascular hyperplasia with subsequent inflammation
  • Increased nucleoli and intranuclear inclusion bodies seen in osteoclasts on microscopy

Pediatric Considerations
Generally not seen in children

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Citation

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TY - ELEC T1 - Paget Disease ID - 307499 Y1 - 2016 PB - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307499/all/Paget_Disease ER -