- Named after Sir James Paget (1814-1899)
- First described osteitis deformans in 1877
- Aetiology is unknown - possibly due to a viral infection
- Often an incidental finding on x-ray in an asymptomatic patient
- If symptomatic usually causes bone pain
- Clinical signs include characteristic skull and long bone deformities
Complications
- Pathological fractures - complete or incomplete
- Neurological effects
- Cranial nerve lesions
- Spinal cord lesions
- Osteoarthritis
- Sarcomas
- Cardiac failure
Histological features
- Osteoclasts are enlarged
- Increased bone turnover produces a mosaic pattern of lamellar bone
- Three phases recognised - osteolytic, mixed and sclerotic
Radiological features
- Osteolytic phase can produce osteoporosis circumscripta
- Bone softening can produce bowing, platybasia, protrusion acetabuli or greenstick fractures
- Mixed phase shows generalised bone enlargement
- Sclerotic phase shows increased density, trabeculae and cortical thickening
Biochemistry
- Serum calcium and phosphate are usually normal
- Serum alkaline phosphatase is increased
- Uric acid increased in about 30% of patients
Treatment
- Non-steroidal anti-inflammatory agents will control bone pain
- Biphosphonates will reduced bone turnover
- Neurological complications and fractures may require surgical intervention
Paget's sarcomas
- Most osteosarcomas that develop lat in life are associated with Paget's disease
- Malignant change occurs in less than 1% patients with Paget's disease
- 50% are osteosarcomas
- 25% are fibrosarcomas
- 25% are giant-cell sarcomas
- The commonest site is the femur
- Prognosis of Paget's sarcomas is poor
- Median survival is one year
- Only 5% alive at five years
Bibliography
Keen R W. The current status of Paget's disease of bone. Hosp Med 2003; 64:
230-232.
Ooi C G, Fraser W D. Paget's
disease of bone. Postgrad Med J 1997;
73: 69-74.
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