Paget's Disease of the Bone

Question 1

what is paget’s disease?

Answer 1

disease of increased but uncontrolled bone turnover

Question 2

what is the pathophysiology of paget’s disease?

Answer 2

1. excessive oestoclastic resorption
2. increased osteoblastic activity

Question 3

what is paget’s disease a disorder of?

Answer 3

osteoclasts

Question 4

what area of the bone does paget’s disease typically affect?

Answer 4

• skull
• spine/pelvis
• long bones of the lower extremities

Question 5

what are the predisposing factors for paget’s disease?

Answer 5

• increasing age
• male sex
• northern latitude
• family history

Question 6

what are the clinical features of paget’s disease?

Answer 6

• older male
• bone pain
• isolated raised ALP

Question 7

what signs are seen if paget’s disease goes untreated?

Answer 7

• bowing of tibia
• bossing of skull

Question 8

what percentage of patients with paget’s disease are symptomatic?

Answer 8

5%

Question 9

what blood tests are done in paget’s disease?

Answer 9

• raised ALP
• normal calcium
• normal phosphate

Question 10

what is seen on x-ray in paget’s disease?

Answer 10

oestolysis in early disease –> mixed lytic/sclerotic lesions later

Question 11

what is seen on skull x-ray in paget’s disease?

Answer 11

• thickened vault
• osteoporosis circumscripta

Question 12

what is seen on bone scintigraphy in paget’s disease?

Answer 12

increased uptake seen focally at the sites of active bone lesions

Question 13

what is the indications for treatment for paget’s disease?

Answer 13

• bone pain
• skull or long bone deformity
• fracture
• periarticular pagets

Question 14

how is paget’s disease managed?

Answer 14

biphosphonates
oral risedronate or IV zoledronate

Question 15

what are the possible complications from paget’s disease?

Answer 15

• deafness
• bone sarcoma
• fractures
• skull thickening
• high-output cardiac failure

Question 16

what is the cause of deafness in paget’s disease?

Answer 16

cranial nerve entrapment