Paget's disease of the bone

Question 1

What is paget’s disease of bone?

Answer 1

A chronic, progressive bone disorder that is characterised by focal areas of increased bone remodelling, resulting in overgrowth of poorly organised bone

Question 2

What areas does Paget’s affect?

Answer 2

• Pelvis (70%)
  
  • Femur (55%)
  • Lumbar spine (53%)
  • Skull (42%)
  • Tibia (32%)
  • Sacrum

Question 3

What risk factors for Pagets?

Answer 3

Age > 55
Male
FH
Smoking
Paramyxoviruses

Question 4

What are Sx of paget’s?

Answer 4

Bone pain (at night ,dull, boring)

Hearing loss
Radiculopathy
Pathological fractures
Bony deformities
Bowed tibia, shortening of legs

Often Asx.

Question 5

What are differentials for paget’s/

Answer 5

• Fibrodysplasia of the bone
• Osteomalacia (25 OH vitamin D would be low)
Rare genetic bone conditions e.g. familial hyperphosphatemia

Question 6

What Ix in Paget’s?

Answer 6

Bloods: ALP may be raised or normal. Do bone specific ALP w PINP and CTX (markers of formation and resorption)

Ca, PO4, vit D.

X-ray: shows lytic lesions, deformity, sclerosis

Biopsy is definitive, but can increase fracture risk

Question 7

What is Rx of Paget’s?

Answer 7

Bisphosphonates or calcitonin if Sx/high riskl.

Observe asymptomatic

Surgery if complications.

Give vit D and calcium if deficient

Analgesia

OT, PT

Question 8

What are complications of paget’s?

Answer 8

Nerve root compression e.g. hearing loss
Spinal stenosis 
OA and fractures
Osteosarcoma 
HF