Paget's Disease of Bone

Question 1

What is Paget’s disease of bone characterised by

Answer 1

Increased bone turnover

Question 2

Most patients of Paget’s disease are extremely symptomatic. True or False

Answer 2

False - most are asymptomatic

Question 3

What sex are affected more with Paget’s disease

Answer 3

Men and women are affected equally

Question 4

In what areas of the world is Paget’s disease more common

Answer 4

Areas of the world with large concentrations of people of Anglo-saxon origin
Rare in asia, africa and scandinavia

Question 5

What results in an abnormal “mosaic” pattern of lamellar bone

Answer 5

Excessive bone resorption by abnormally large osteoclasts followed by increased bone formation by osteoblasts in a disorganised fashion

Question 6

How is a diagnosis of Paget’s disease obtained

Answer 6

Plain radiograph for some other reason

or Biochemistry for some other reason showing raised alkaline phosphatase

Question 7

Where are the most commonly affected areas of Paget’s disease

Answer 7

pelvis
spine
skull
long bones (proximal and distal)

Question 8

can Paget’s disease spread from one bone to the next

Answer 8

No - the distribution within their skeleton is likely to remain fixed

Question 9

What might pain be due to in Paget’s disease

Answer 9

periosteal stretching caused by bone enlargement, micro fractures or secondary degenerative arthritis

Question 10

What might some patients complain of

Answer 10

headache due to skull involvement

Question 11

Describe the changes seen in Paget’s

Answer 11

enlarged and abnormally contoured bones e.g. anterior bowing of the tibia and anterolateral bowing of the femur

Question 12

What are fractures sometimes associated with

Answer 12

substantial acute blood loss

Question 13

Patients with Paget’s have a 1% increase in developing what

Answer 13

osteosarcoma

Question 14

What is a marker of increased bone formation

Answer 14

serum alkaline phosphatase

Question 15

What feature may be seen in the early stages of Paget’s disease

Answer 15

Lytic lesions

Question 16

What does chaotic bone formation result in that can be seen on radiograph

Answer 16

lack of distinction between the cortex and medullary bone

Question 17

What feature is helpful in differentiating Paget’s disease from sclerotic metastases

Answer 17

The affected bones are expanded but in sclerotic metastases, the bone is of normal size

Question 18

What causes a cotton wool appearance

Answer 18

disruption of normal bone architecture

Question 19

What is useful in determining the extent of skeletal involvement, but is not specific for diagnosis

Answer 19

Radioisotope bone scanning

Question 20

What are Paretic bone lesions seen as

Answer 20

hot spots

Question 21

What is the primary indication for treatment in Paget’s disease

Answer 21

presence of symptoms

Question 22

What should be done if there is doubt about where the pain is coming from in a Patient with both osteoarthritis and Paget’s disease

Answer 22

Prescribe NSAIDs and paracetamol

Question 23

What do drugs used to treat Paget’s disease do

Answer 23

suppress osteoclastic activituy

Question 24

Why are bisphosphonates used as first line

Answer 24

Superior efficacy compared with calcitonin and their minimal side effects

Question 25

What bisphosphonate is the drug of choice and how is it administered

Answer 25

IV pamidronate may be given fortnightly

This usually suppresses disease activity for 12-18 months

Question 26

What are some side effects of pamidronate

Answer 26

Low grade fever and flu-like symptoms in the first 1-2 days in 20% of patients
Hypocalcaemia
Rare side effects include ocular complications (conjunctivitis, uveitis, scleritis) and osteonecrosis of the jaw

Question 27

What is the main down side of using oral risedronate

Answer 27

may cause oesophagi’s

Question 28

How is calcitonin administered

Answer 28

subcutaneously at bedtime

the dose is then reduced and only given every other day

Question 29

What is the disadvantage of calcitonin

Answer 29

patients become resistant due to the development of antibodies

Question 30

What are some side effects of calcitonin

Answer 30

Nausea
facial flushing
metallic taste

Question 31

What type of surgery is sometimes beneficial for patients with refractory pain

Answer 31

Elective surgery for joint replacement
tibial osteotomy
internal fixation of pathological fractures

Question 32

What should be measured initially every other month and then once or twice a year with good clinical response

Answer 32

serum alkaline phosphatase

Question 33

When are repeat bone scans and radiographs not necessary

Answer 33

Never unless the patient has new or progressive symptoms