Paget's Disease

Question 1

what is paget’s disease?

Answer 1

disease of increased, uncontrolled bone turnover
primarily disorder of OC - excessive resorption followed by increased OB activity

Question 2

prevalence of paget’s?

Answer 2

uk prev is 5%
symptomatic only in 1 in 20

Question 3

what areas are affected in paget’s (in order)?

Answer 3

spine
skull
pelvis
femur

Question 4

predisposing factors for paget’s?

Answer 4

increasing age
male
northern latitude
family history

Question 5

clinical features of paget’s?

Answer 5

older male with bone pain + isolated raised ALP
bone pain
classical untreated features - bossing of skull, bowing of tibia

Question 6

ix for paget’s?

Answer 6

bloods - raised ALP, normal ca and phosph
other markers of bone turnover
x-rays - abnormal thickened + sclerotic bone
bone scintigraphy - increased uptake focally at sites of active bone lesions

Question 7

when may ca be raised in paget’s?

Answer 7

prolonged immobilisation

Question 8

what are other markers of bone turnover other than alp/ca/phosph?

Answer 8

procollagen type 1 n-terminal propeptide
serum c-telopeptide
urinary n-telopeptide
urinary hydroxyproline

Question 9

what is seen on x-rays in paget’s?

Answer 9

osteolysis in early disease to mixed lytic/sclerotic lesions later

skull x-ray shows thickened vault, osteoporosis circumscripta

Question 10

mx of paget’s?

Answer 10

treat if: bone pain, skull/long bone deformity, fracture, periarticular paget’s

oral bisphosphonate (po risedronate, else iv zoledronate)
calcitonin less common now

Question 11

comps of paget’s?

Answer 11

bone sarcoma
high-output cardiac failure
deafness (cranial nerve entrapment)
fractures
skull thickening