Pagets Disease

Question 1

Pagets disease defintion

Answer 1

Defined as a condition of disorganised bone remodelling

Question 2

Epidemiology

Answer 2

• second most common bone disease
• highest prevalance in the UK
• males > females

Question 3

Pathology

Answer 3

• Excessive bone resporption (osteolysis) followed by weak, disorgansied abnormal bone being rapidly laid down
• primary abnormality is within osteoclast - increased number and size, multinucelated
• normal osteoblasts with increased activity
• bone architecturally abnormal and has reduced mechanical strength

Question 4

Clinical features

Answer 4

• Often asymptomatic
• 10-30% have symptoms: PANICS
  
  • Bone Pain/ Patholgical fracture
  • Arthlagia
  • Nerve root compression/ Neural deafness
  • increased bone density
  • Cardiac failure
  • Skull/Sclerotic vertebrae
• Can be single or multiple sites

Question 5

Bones affected

Answer 5

• pelvis
• lumbar spine
• femur
• throacic spine
• sacrum
• skull
• tibia
• humerus

Question 6

Investifation

Answer 6

• Bone profile - raised ALP
• bone turnover markers increased
• X-ray - thickened coarse bone, osteoporosis circumscripta, pathological fractures

Question 7

Treatment

Answer 7

• treatment indicaitons include- bony pain, deformity and diseases sequelale (fractures, nerve compression)
• bisphosphanates
• surgery