Paget's Disease

Question 1

What is the stereotypical presentation of Paget’s disease of the bone?

Answer 1

older male with bone pain + an isolated raised ALP

Question 2

Give 2 features of untreated Paget’s disease

Answer 2

bowing of tibia
bossing of skull

Question 3

Describe the epidemiology of Paget’s disease

Answer 3

Common (UK prevalence 5%)
but symptomatic in only 1 in 20 patients

Question 4

Give 4 risk factors for Paget’s disease

Answer 4

Age
Male
Northern latitude
FH

Question 5

Which bones are usually affected in Paget’s disease?

Answer 5

Skull
Spine
Pelvis
Long bones of lower limbs

Question 6

What blood profile is usually seen in Paget’s disease?

Answer 6

High ALP
Normal Calcium + Phosphate

(Hypercalcaemia may occasionally occur with prolonged immobilisation)

Question 7

What is seen on X-ray in Paget’s disease?

Answer 7

Osteolysis in early disease → mixed lytic/ sclerotic lesions later

Skull x-ray: thickened vault, osteoporosis circumscripta

Question 8

What is seen on bone scintigraphy in Paget’s disease?

Answer 8

increased uptake focally at sites of active bone lesions

Question 9

What is the management of Paget’s disease?

Answer 9

Bisphosphonate: Risedronate PO or Zoledronate IV

Question 10

List 5 complications of Paget’s disease

Answer 10

Deafness (cranial nerve entrapment)
Bone sarcoma (1% if affected for > 10y)
Fractures
Skull thickening
High-output cardiac failure

Question 11

How must Aldendronate be taken?

Answer 11

at least 30 mins before breakfast with plenty of water + sit-upright for 30 mins following
(poor oral bioavailability, furher reduced by food/ drink)