Rheumatology - Paget's Disease

Question 1

What is Paget’s disease of the bone?

Answer 1

Excessive bone turnover due to increased osteoclast and osteoblast activity

Question 2

What happens as a result of Paget’s disease of the bone?

Answer 2

Excessive turnover not coordinated

Causes patchy areas of sclerosis and lysis

Enlarged and misshapen bones, structural problems and increased risk of pathological fractures

Question 3

What part of the skeleton is primarily affected in Paget’s disease of the bone?

Answer 3

Axial skeleton

Question 4

How does Paget’s disease of the bone present?

Answer 4

Bone pain
Bone deformity
Fractures
Hearing loss

Question 5

What investigations are used for Paget’s disease of the bone?

Answer 5

X-ray
Blood tests

Question 6

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

Answer 6

Bone enlargement and deformity
Osteoporosis circumscripta
(well-defined osteolytic lesions that appear less dense compared to normal bone)
Cotton wool appearance of the skull
V-shaped osteolytic
(defects in long bones)

Question 7

What do blood tests show in Paget’s disease of the bone?

Answer 7

Raised ALP
Normal calcium
Normal phosphate

Question 8

How is Paget’s disease of the bone managed?

Answer 8

Bisphosphonates
Calcitonin if bisphosphonates contraindicated
Analgesia
Calcium and vitamin D
Surgery if severe deformities

Monitor serum ALP

Question 9

What are the key complications of Paget’s disease of the bone?

Answer 9

Hearing loss (if ossicles affected)
Heart failure
Osteosarcoma
Spinal stenosis and spinal cord compression