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  • • Also known as osteitis deformans

    • Nonmalignant disease involving accelerated bone resumption followed by deposition of dense, disorganized, and ineffectively mineralized bone matrix

    • Etiology is unknown, although posited to be infectious; hereditary causes possible as well

    • 3 phases of disease:

    • -Intense bone resorption

      -Production of abundant woven bone with ineffective mineralization

      -Deposition of sclerotic, chaotic cortical and trabecular bone

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Epidemiology

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  • • Second most common bone disorder, behind osteoporosis

    • Male:female ratio is 1:1

    • Affects 3% of persons in the United States

    • Affects 10% of persons older than 80 years

    • Up to 40% of patients have positive family history for Paget disease

    • 1-10% malignant degeneration of pagetic bones

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Symptoms and Signs

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  • • Pain in the affected bone

    • Alteration in hearing and vision

    • Loosening of teeth (if jaw involvement)

    • Headaches

    • Damage to cartilage, with resultant arthritis

    • Nephrolithiasis

    • High output heart failure (from AV shunting in bone marrow)

    • Kyphosis

    • Frontal bossing

    • Leonine facies

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Laboratory Findings

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  • • Elevated serum calcium

    • Elevated serum alkaline phosphatase

    • Elevated urine calcium

    • Elevated urinary pyridinoline

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Imaging Findings

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  • • Soft-tissue masses and cortical breakthrough on plain x-rays suggest malignant transformation

    • Radiographs include both early lytic lesions and late sclerotic findings

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  • • Always consider metastatic disease to bone

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  • • Complete history (including family) and physical exam

    • Appropriate x-rays

    • Serum calcium, alkaline phosphatase, and urinary pyridinoline

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  • • Treatment does not cure but prolongs periods of remission

    • Goals should include aggressive pain control

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Surgery

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Indications

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  • • Progressive bowing of the tibia or femur

    • Delayed union of fractures

    • Unstable fractures

    • Arthritis refractory to medical treatment

    • Focal nerve compression of the spine or cranium

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Medications

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  • • Bisphosphonates inhibit osteoclast resorption

    • Injectable calcitonin

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Treatment Monitoring

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  • • Repeat radiographs, especially of weight bearing joints, to monitor degeneration

    • Serum alkaline phosphatase (from every 3 to 12 months)

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Complications

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  • • Malignant degeneration into osteosarcomas, fibrosarcomas, or undifferentiated spindle cell sarcomas

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References

Schneider D et al. Diagnosis and treatment of Paget's disease of bone. Am Fam Physician. 2002;65:2069.  [PubMed: 12046775]
Hullar TE, Lustig LR. Paget's disease and fibrous dysplasia. Otolaryngol Clin North Am. 2003;36:707.  [PubMed: 14567061]

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