Paget's disease of bone

Paget's disease is the second most common metabolic bone disease after osteoporosis and is the commonest cause of bone dysplasia.

• it is characterised by one or more areas of aggressive osteoclast-mediated bone resorption followed by imperfect osteoblast-mediated bone repair (1)

Involvement of the disease is monostotic in 15% of cases and polyostotic in the remainder.

• can affect any bone but frequently the axial skeleton is involved. The usual sites include
  • pelvis (70%)
  • femur (55%)
  • lumbar spine (53%)
  • skull (42%)
  • tibia (32%) (2)
• when long bones are affected they may become bowed
• thickening of the skull may lead to compression of cranial nerves with consequent palsies.

Many cases of Paget's disease are asymptomatic.

Reference:

• (1) Whyte MP. Clinical practice. Paget's disease of bone. N Engl J Med.2006;355(6):593-600.
• (2) Langston AL, Ralston SH. Management of Paget's disease of bone. Rheumatology (Oxford). 2004;43(8):955-9.