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Paget’s Disease

Paget's disease is where there's 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. It usually affects the skull, spine, pelvis, tibia, or femur.


Risk factors - Increasing age (> 40 years), Family history


Presentation

Is often asymptomatic, but symptoms may include:

  • Bone pain and deformity

  • Pathological fractures


Investigations and Management

XR - This may show:

  • 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

“Paget's disease of right hip bone in an 80-year-old man” © Jmarchn CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0/)

Bloods - Raised ALP, but Calcium and Phosphate will be normal


Management:

  • Analgesia

  • Bisphosphonates e.g. Alendronate, Zoledronic acid

    • Side-effects - Reflux, Oesophageal erosions, Jaw osteonecrosis


Complications

  • 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 cancer with a very poor prognosis




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