Paget's disease of bone

Osteitis Deformans

  • D: Second most common metabolic bone disease (first is osteoporosis) characterised by excessive / disorganised bone formation and resorption

  • I/A: Increasing incidence with age - 3-4% over 40 years, 10% 85+
  • S: More common in men than women
  • G: More common in the west, less common in east asian countries
  • A: Unknown but though to be persistent infection in bone from a virus (Measles, mumps, RSV)
  • P: Alternating phases of rapid bone resorption/formation
  • M: Long bones have thicked shafts/deformities, cysts and stress fractures seen. May be areas of localised softening - "osteoporosis circumscripta".
  • M: (1) Osteolytic phase - clasts produce excavations which are filled with vascular fibrous tissue (2) Mixed lytic-sclerotic phase - blasts lay down woven bone which is subsequently resorbed by osteoclasts (3) Quiescent osteosclerotic stage - clasts less active, eroded areas filled with brittle woven bone
  • P

Clinical features

  • Usually asymptomatic
  • Bone: 15% monostotic, 85% polyostotic
  1. Long bones - become bowed/deformed
  2. Cranium - compression of cranial nerves as pass through foramina (8th - leading to deafness), compression of nerve roots (causing pain)
  3. Pathological fractures
  4. Osteosarcoma (1%)
  • Cardiac
  1. High output cardiac failure - most common in polyostotic disease (increased bone blood flow)

Investigations

  • Radiology
  • Increased ALP (reflects osteoblastic activity)
  • Plasma calcium normal/raised

Treatment

  • Control pain
  • Suppress bone turnover - bisphosphonates, serum ALP for measuring disease progression
  • Surgery - pathological fractures, joint replacement, nerve/cord decompression