Paget’s disease

Paget’s disease is caused by increased bone remodelling by osteoclasts and osteoblasts. This results in in bone enlargement, pain, deformity and weakness. The cause is unknown.

Epidemiology

• 1-2% of white >55 year olds
• High incidence in UK; low incidence in Asia

Investigations

• Imaging: plain X-rays (bone enlargement and ‘cotton wool’ lytic/sclerotic pattern), bone scan (scintigraphy/technetium scan)
• Blood tests: ALP (increased), calcium/phosphate/vitamin D (all normal) 
• Other tests: urine/serum hydroxyproline, urine pyridinoline, serum procollagen I N-terminal peptide (PINP)

Clinical features

Symptoms

• Bony pain
• Pathological fractures 

Signs

• General inspection
  • Kyphosis
  • Paraplegia (spinal disease) 
  • Bone pain
• Hands inspection
  • Osteoarthritis
• Face
  • Frontal bossing of skull
  • Nerve deafness and tinnitus (due to bony compression of CN8)
  • Enlarged maxilla
• Neck
  • Raised JVP (heart failure due to hyperdynamic circulation) 
• Legs
  • Bowed femur
  • Sabre (bowed) tibia
  • Feel warm
  • Pathological fractures
  • Osteoarthritis

Management

• Analgesia (e.g. NSAIDs and paracetamol)
• Bisphosphonates (e.g. zoledronic acid)
• Ensure adequate vitamin D and calcium intake
• Physiotherapy and orthotic devices

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