Paget's disease of the bone

Question 1

What is Paget’s?

Answer 1

Disease of increased but uncontrolled bone turnover

Primarily disorder of osteoclasts

Question 2

Which bones are commonly affected?

Answer 2

Skull, spine/pelvis and long bones of the lower extremities

Question 3

Predisposing factors of Paget’s

Answer 3

increasing age
male sex
northern latitude
family history

Question 4

Clinical features

Answer 4

the stereotypical presentation is an older male with bone pain and an isolated raised ALP
bone pain (e.g. pelvis, lumbar spine, femur)
classical, untreated features: bowing of tibia, bossing of skull
raised alkaline phosphatase (ALP) - calcium* and phosphate are typically normal
other markers of bone turnover include: procollagen type I N-terminal propeptide (PINP), serum C-telopeptide (CTx), urinary N-telopeptide (NTx), and urinary hydroxyproline
skull x-ray: thickened vault, osteoporosis circumscripta

Question 5

Indications for treatment in Paget’s

Answer 5

• Bone pain
• Skull or long bone deformity
• Fracture
• Periarticular Paget’s

Question 6

Treatment of Paget’s

Answer 6

Bisphosphonate- oral risedronate or IV zoledronate

Calcitonin

Question 7

Complications of Paget’s

Answer 7

deafness (cranial nerve entrapment)
bone sarcoma (1% if affected for > 10 years)
fractures
skull thickening
high-output cardiac failure