Paget's Disease

Question 1

What is it?

Answer 1

Disorder of bone turnover
Excessive bone turnover due to excessive osteoblastic and osteoclastic activity
Turnover is not coordinated properly- patchy areas of high density and low density
Causes enlarged misshaped bones that fracture easily

Question 2

What are the symptoms?

Answer 2

Up to 80% are asymptomatic
Bone pain
Bone deformity
Fractures
Cranial nerve palsies esp VIII- hearing loss

Question 3

What Investigations are needed?

Answer 3

Bloods

• ALP= raised
• CA= mildly raised in widespread disease

Urine
-raised hydroxyproline (component of collagen) due to increased bone turnover

XR
-Bone enlargement and deformity
-Osteoporosis circumscripta- well defined osteolytic lesions that appear less dense than normal bone
Cotton wool appearance due to sclerosis and lysis
V-shaped defects

Bone Scans

• to assess bone involvement
• cannot differentiate between PDB and sclerotic mets

Question 4

What is the management?

Answer 4

Analgesia for pain e.g. NSAIDs
Bisphosphonates are mainstay
-improve symptoms and prevent further changes
-restores normal bone metabolism

Surgery for secondary joint disease/neurological complications

Must monitor ALP- effective treatment should normalise it

Question 5

What are the complications?

Answer 5

Osteogenic sarcoma

Spinal stenosis and spinal cord compression