Paget’s Disease/Osteitis deformans

Wikis > General Medicine > Bone Disorders > Paget’s Disease/Osteitis deformans

Paget’s Disease/Osteitis deformans:

Bone remodelling disease of unknown cause – often asymptomatic. Characterised by aggressive one resorption by osteoclasts and formation by osteoblasts  thickened bone that is structurally weak  complications.
Radiographically present in up to 3% of population.

Most commonly affects pelvis, sacrum, spine, skull, femur, tibia, fibula, calcaneus

Aetiology
Unknown, but could be inflammatory (does respond to corticosteroids – but little other evidence); vascular (maybe an autonomic dysfunction); viral (intranuclear bodies are found in osteoclasts indicating a virus); collagen defect; genetic; benign neoplasm; vitamin D deficiency. No known HLA associations. 7x more common in relatives.

Clinical features
Present with wide spectrum of symptoms – bone pain (80%); pathological fracture; neurological symptoms (from bone enlargement and deformity); joint symptoms (50%) and joint destruction.
40% associated with hyperuricaemia.

Involvement of feet and hands is less common than other bones.

Laboratory:
Elevated serum alkaline phosphatase

Differential diagnosis:
Hyperparathyroidism, osteosclerosis, healing traumatic fracture, neoplasm, osteomyelitis, osteomalacia

Radiological
Cortical thickening, lytic lesions
Increased focal uptake on bone scan

Stages
1) Abnormal destruction of bone
2) Formation of woven bone
3) Waves of bone resorption and formation
4) Intense sclerosis

Management:
Drugs – calcitonin (inhibits bone resorption, analgesic); bisphosphonates (decrease bone resorption and formation); mithramycin (cytotoxic); analgesics
Surgical for fractures and deformity

Podiatric implications:
Involvement of foot –
Podiatric management – management of deformity  hyperkeratosis.

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