Paget's Disease of the Bone

Question 1

What is Paget’s disease of the bone?

Answer 1

A focal disorder of bone remodelling.

Increased osteoclast bone resportion
Compensatory increase in bone formation
Disorganised mosaic of woven and lamellar bone

Question 2

What is the epidemiology of Paget’s disease?

Answer 2

4% individuals over 40
Genetic predisposition (15-30%)
Ethnic and geographic clustering

Question 3

What are the clinical features of Paget’s disease?

Answer 3

Common:
Pain
Deformity
Fracture 
LOCALISED

Rare:
Deafness
Myelopathy (Spinal cord compression, vascular steal)
Secondary osteoarthritis 
Osteosarcoma
Hypercalcaemia

Question 4

What are the investigations?

Answer 4

X-ray
Biochemistry (High alkaline phosphatase, due to high turnover but Calcium and Phosphate normal as localised disease)
Isotope to assess extent (high uptake in affected regions - commonly asymmetrical)

Question 5

What are the x-ray findings of Paget’s disease?

Answer 5

Expansion
Cortical thickening
Abnormal texture

Question 6

What is the treatment?

Answer 6

Bisphosphonates
(usually only if active and symptomatic)
Surgery to treat complications

Question 7

How would you identify active disease?

Answer 7

High alkaline phosphatase

Normal calcium and phosphate

Question 8

What is the relationship between radiological evidence and symptoms?

Answer 8

Radiologically very common

Minority symptomatic

Question 9

What is the classic triad of symptoms?

Answer 9

Pain, deformity and fracture