Pagets

Question 1

What is Paget’s disease of the bone?

Answer 1

uncontrolled bone turnover

excessive uncoordinated bone turnover (formation and resorption) due to excessive activity of mainly osteoclasts and also osteoblasts . Leads to patchy areas of high density (sclerosis) and low density (lysis)

results in bone enlargement and deformity causing structural problems = > risk of fractures

Question 2

Which bones does paget’s disease especially effect?

Answer 2

1. Axial skeleton - bones of the head and spine
2. Pelvis
3. Lone bones of the lower extremities

Question 3

How does Pagets disease present?

Answer 3

stereotypical: older male with bone pain and isolated ALP

1. Bone pain
2. Bone deformity - classical: bowing of tibia, bossing of skill (if left untreated)
3. Raised ALP
4. Other features of bone turnover: PINP, CTx, NTx, urinary hydroxyproline

Question 4

What are the potential complications of Paget’s disease?

Answer 4

• deafness (cranial nerve entrapment)
• Bone sarcoma (1% if affected for >10 years)
• Fractures
• Skull thickening

Question 5

What are the risk factors for Paget’s disease

Answer 5

increasing age
male sex
northern latitude
family history

Question 6

What investigations would you do?

Answer 6

1. X-ray
2. ALP - raised (other LFTs normal)
3. Calcium (normal)
4. Phosphate (normal)

Question 7

What findings would you see on X-ray?

Answer 7

1. bone enlargement and deformity
2. Osteoporosis Circumscripta - well defined osteolytics lesions, appear less dense than darker bone
3. Cotton wool appearance of skull (lytic and sclerotic regions)
4. skull thickening

Question 8

What is the management of Paget’s Disease?

Answer 8

Bisphosphonates (Risedronate or IV Zoledronate)