Paget's disease

Question 1

What is Paget’s disease of the bone?

Answer 1

Disorder of excessive bone turnover due to excessive activity of both osteoblasts and osteoclasts

Question 2

What is the pathology in Paget’s disease of the bone?

Answer 2

Excessive osteoblast and osteoclast activity leads to areas of sclerosis and of lysis

Increased risk of pathological fractures

Question 3

Presentation of Paget’s disease of the bone

Answer 3

The stereotypical presentation is an older male with bone pain and a raised ALP

• Bone pain
• Bone deformity
• Fractures
• Hearing loss can occur if it affects the bones of the ear

Most people are asymptomatic

Question 4

Predisposing factors for Paget’s disease of the bone

Answer 4

Increasing age
Male sex
Northern latitude
Family history

Question 5

Investigations for suspected Paget’s disease of the bone

Answer 5

X-ray - may see

• Bone enlargement and deformity
• Mixed osteolytic and sclerotic lesions (often just osteolytic in early disease)

Bloods - FBC, Bone profile, LFTs

• LFTs - isolated raised ALP (other LFTs normal)
• Normal calcium
• Normal phosphate

Question 6

Management of Paget’s disease of the bone

Answer 6

Bisphosphonates are the main treatment

Others:

• NSAIDs for bone pain
• Calcium and vitamin D supplementation particularly whilst on bisphosphonates
• Surgery rarely required for fractures, severe deformities

Monitoring:
- Serum ALP and reviewing Sx

Question 7

Complications of Paget’s disease of the bone

Answer 7

Osteogenic sarcoma (osteosarcoma)

• Bone cancer with poor prognosis
• Increased risk of this in Paget’s disease
• Presents with focal bone pain, swelling or pathological fractures

Spinal stenosis and spinal cord compression

• Diagnose with MRI
• Treat with bisphosphonates
• Consider surgery