Paget's disease of bone

Background

• Characterized by areas of bone with excessive osteolytic activity and subsequent increased ostoblastic activity, resulting in disorganized bone formation
• Results in osteolytic lesions and weakened bone
• Eventually, affected bones may be abnormally enlarged
• Exact etiology unknown, likely genetic component
• Prevalence increases with age, more common in people of European descent

Clinical Features

• Initially asymptomatic or mild pain
• Bone pain at affected sites, often worse at night
• Pathologic fractures
• If skull involved, may report increased hat size, headache, decreased hearing

Differential Diagnosis

• Bone tumor (e.g. osteosarcoma or metastesis)
• Multiple myeloma, myelofibrosis
• Fibrous dysplasia
• Intramedullary osteosclerosis
• Sickle cell disease

Evaluation

• Imaging to evaluate for fractures
  • Initial lesion may be destructive, radiolucent (especially in skull)
  • Involved bones may be expanded, abnormally dense
  • May have multiple fissure fractures in long bones
• Alk phos typically very high
• Serum calcium may be high

Management

• Treat fractures
• Pain control
• Bisphosphonates (e.g. Zoledronate) are treatment of choice for patients with severe symptoms or advanced disease

Disposition

• Discharge uncomplicated patients