Paget's Disease
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-525-6884
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Sir James Paget, a prolific surgeon and pathologist, described several diseases, including:
- Paget's disease of bone (most common use of Paget's disease)
- Paget's disease of the breast
- Paget-Schroetter disease
- Extramammary Paget's disease (EMPD)
- Paget's disease of the penis
- Paget's disease of the vulva
Overview
- Paget's Disease of bone is typically focal
- It is characterized by excessive resoprtion of bone by osteoclasts followed by the rapid and irregular deposition of new bone
Epidemiology and Demographics
The incidence of
- Likelihood of occurrence increases with age
- Radiologic surveys show the frequency in adults is less than 1% in the US, Great Britain, and Australia
- The disease is rare in India, Japan, Scandanavia, and the Middle East
Risk Factors
- Not known
Pathophysiology & Etiology
- The cause of Paget's Disease is unknown
- The early phase of the disease is termed the destructive phase of disease
- The next phase, commonly termed the mixed phase, involves the formation of new pagetic bone.
In the next phase, called the scelrotic phase, the resorptive rate declines relative to the rate of bone formation leading to the development of less vascular bone and a positive calcium balance.
Diagnosis
Differential Diagnosis
Since many patients are asymptomatic, the disorder is commonly discovered by radiologic examination for an unrelated disease or because of an elevated plasma alkaline phospatase.
History and Symptoms
The disease is localized and generally does not cause symptoms
- Some patients may notice swelling or a deformity in a long bone
- Enlargement in the skull can occur, though it is generally not noticed by patients
- Headache, facial pain, backaches, and leg pain are commonly reported
- Back pain is typically in the lower back and buttocks
- Hearing loss can occur due to involvement of the ossicles of the inner ear or of bone in the region of the cochlea
- Some serious neurologic complications can arise due to overgrowth of bone near the base of the skull
- Paraplegia can result from compression of the spinal cord, especially in the middorsal spine
Laboratory Findings
Electrolyte and Biomarker Studies
There is an elevation of the alkaline phosphatase
Treatment
Most patients do not require treatment since the disease is generally asymptomatic
Pharmacotherapy
Chronic Pharmacotherapies
Cytotoxic drugs like plicamycin and dactinomycin are no longer used for therapy
- Bisphosphonates are used to inhibit bone resorption
- Etidronate has been used at 20 mg/kg body weight per day and is effective in producing clinical improvement
- osteomalacia is a potential complication of therapy
- Alendronate, pamidronate, risendronate, and tiludronate are the bisphosphonates most commonly used now as they are more potent than etidronate and do not result in the same mineralization defects
- Alendronate is approx. 700-fold more potent than etidronate
- Alendronate (oral administration) and pamidronate (intravenous administration) are approved for use in the US
Common bisphosphonates and their use:
- Alendronate is administered orally with water 30-60 minutes before breakfast after an overnight fast
- Dose is 40 mg/d for 6 months
- Pamidronate is given intravenously
- Dose is 30 mg/d in 5% glucose in water or normal saline over 4h ion 3 successive days
- Calcitonin will be replaced by bisphosphonates for primary treatment of severe disease, but calcitonin can still be used for patients who cannot tolerate alendronate due to gastrointestinal side effects
- The administration of calcitonins suppresses the pagetic lesion which leads to a decrease in bone pain.
- Calcitonin also improves neurologic symptoms and decreases elevated cardiac output
- Some individuals do not respond to porcine or salmon calcitonins
- Calcitonin can be administered by nasal spray at doses of 200 IU/d.
References
Acknowledgements
The content on this page was first contributed by Shaun Takao, C. Michael Gibson, M.S., M.D.
List of contributors:
Suggested Reading and Key General References
Suggested Links and Web Resources
For Patients
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
