Paget’s Disease (1)

Question 1

What’s its pathophysiology?

Where does it usually affect?

What are its risk factors?

Answer 1

➊ • Increased, uncoordinated bone turnover due to excessive osteoblast and osteoclast activity
• This leads to patchy areas of high density (Sclerosis) and low density (Lysis) = Enlarged, misshapen bones, which increases the risk of pathological fractures

➋ Skull, spine, pelvis, tibia, femur

➌ • Increasing age (>40 yrs)
• Family history of Paget’s Disease

Question 2

How does it present?

What are the key complications that can occur?

Answer 2

➊ Often asymptomatic, but symptoms include:
• Bone pain and deformity
• Pathological fractures

➋ • Pathological fractures
• Spinal stenosis
• Nerve compression - Bone overgrowth pressing on nerves - Diagnosed w/MRI and treated with bisphosphonates
• Hearing loss - due to nerve compression or ossicle ossification (most common complication)
• OA
• Osteogenic Sarcoma - Rare bone ca. with a very poor prognosis

Question 3

Investigations and Management:
What is the main investigation to do?
→ What features will be seen?

Which bloods should be done?
→ How can it be differentiated from Osteomalacia here?

How is it managed?
→ What are its main SEs?

Answer 3

➊ XR
→ • Bone enlargement and deformity
• Osteoporosis Circumscripta - Well-defined osteolytic lesions that appear less dense to normal bone
• Cotton wool appearance of the Skull - Poorly-defined patchy areas of high density (Sclerosis) and low density (Lysis)
• Pathological fractures

➋ • ALP (Raised)
→ Calcium and Phosphate will be normal

➌ • Analgesia
• Bisphosphonates e.g. Alendronate, Zoledronic acid
→ Reflux, Oesophageal erosions, Jaw osteonecrosis