Paget's disease

Question 1

Define:

Answer 1

Excessive bone remodelling in one or more sites

This is due to an increase in osetoclasts and osteoblasts which leads to bone enlargement, deformity, weakness and remodelling

Question 2

Aetiology/risk factors:

Answer 2

UNKNOWN

There are believed to be genetic or viral factors

The increase in osteoclasts and osteoblasts leads to abnormally laid down lamellar bone

Marrow spaces are filled with excess fibrous tissue and there is an increase in blood vessels.

Question 3

Epidemiology:

Answer 3

Common in the elderly.

3% in >50 yrs

10% in > 80 yrs

more common in temperate climates and in anglo-saxons.

Question 4

Symptoms:

Answer 4

May be asymp.

Insidious onset - deep boring bone pain (worse on moving and weight-baring areas)

Headaches

weakness

deafness

fractures

increase skull size

Question 5

Signs:

Answer 5

Bitemporal skull enlargement

Spinal kyphosis

Anteolateral bowing of the femur, tibia and forearm

Skin over the affected area may be warm

Sensineural hearing loss

Question 6

Investigations:

Answer 6

Bloods:

• ALP WILL BE VERY HIGH
• CA AND PHOSPHATE WILL BE NORMAL

Bone radiography:

• Bone enlargement + deformity
• lytic + sclerotic bone
• lack of distinction from the medulla and the cortex

Skull changes:

• enlargement of frontal and occipital areas
• cotton wool appearance
• osteoporosis circumspina

Bone scan:

• asses the extent of skeletal involvement
• pagetic bone lesions

resorption markers to monitor the disease