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
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