MSRA: Paget's Disease

Question 1

What is meant by Paget’s disease of the bone?

How does it present clinically?

Answer 1

Condition of abnormal bone remodelling - excessive resorption and abnormal new bone formation
Common sites: femur, pelvis, tibia, skull, spine
Can be described in phases: sclerotic, lytic or mixed

Bone pain, long bone bowing, pathological fractures, large joint OA

Question 2

What are the blood test results in a patient with Paget’s disease of the bone?

Answer 2

Normal calcium
Raised ALP (secondary to increased bone turnover)

Question 3

What are the possible complications of Paget’s disease?

Answer 3

Deafness - due to sclerotic changes within the temporal bone
Heart failure - high vascularity within diseased bone, increases cardiac output
Paget’s sarcoma (less than 1%) malignant secondary sarcoma occurring most commonly in the pelvis, femur and humerus

Question 4

What are the management options in Paget’s disease?

Answer 4

If asymptomatic - observation and supportive therapy (PT, NSAIDs)
If symptomatic: first line bisphosphonates, second line calcitonin

Consider TKR/THR (increased risk of bleeding)
If # secondary to long bone bowing or imminent # - consider metaphyseal osteotomy and plate fixation

Question 5

Why is teriparatide contraindicated in Paget’s disease of the bone?

Answer 5

Associated with an increased risk of secondary osteosarcoma

Question 6

What are the key X-Ray findings in Paget’s disease?

Answer 6

Enlarged, deformed bones
Mixed sclerotic and lytic lesions
‘Cotton Wool’ appearance
Blade of Grass sign