Paget Disease of Bone for Primary Care
Am Fam Physician. 2020 Aug 15;102(4):224-228.
Patient information: See related handout on Paget disease of bone, written by the authors of this article.
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Paget disease of bone is a benign disorder characterized by focal areas of increased bone turnover in one or more skeletal sites. It usually affects older adults, and men are at a higher risk than women. Any bone may be affected, but the disease has a high preference for the pelvis, spine, skull, and long bones. Pain is the most common symptom, and presentation of the disease may depend on which bones are affected, the extent of involvement, and the presence of complications. Paget disease of bone may be asymptomatic, and suspicion arises from incidental findings of elevated serum alkaline phosphatase levels on routine blood work or abnormalities on imaging tests performed for an unrelated cause. Evidence-based guidelines recommend the use of plain radiography and serum alkaline phosphatase testing for initial diagnosis and radionuclide scans for delineation of the extent of disease. Treatment with nitrogen-containing bisphosphonates is recommended in active disease or when risk of complications is possible. Complications of the disease include arthritis, gait changes, hearing loss, nerve compression syndromes, and osteosarcoma. Total serum alkaline phosphatase is the suggested marker for assessing treatment response when high bone turnover occurs, and it should be measured at three to six months to evaluate initial response. Early diagnosis of Paget disease of bone remains key to its management because patients generally have a good prognosis if treatment is initiated before major complications arise. The primary care physician may need to consult with a specialist for confirmation of diagnosis and initiation of treatment.
Paget disease of bone is a benign skeletal disorder characterized by focal areas of increased bone resorption and disorganized bone formation.1 These focal lesions, called pagetic lesions, may be confined to a single site (monostotic) or several sites (polyostotic) of the skeleton. Although any bone may be affected, the pelvis, femur, lumbar spine, thoracic spine, skull, and tibia are most common.2
SORT: KEY RECOMMENDATIONS FOR PRACTICE
A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.
SORT: KEY RECOMMENDATIONS FOR PRACTICE
| Clinical recommendation | Evidence rating | Comments |
|---|---|---|
Patients thought to have Paget disease of bone should have plain radiography of suspicious regions, and those who are asymptomatic with elevated levels of total serum alkaline phosphatase should have plain radiography of the abdomen, skull and facial bones, and tibia.16,18,19 | C | Recommendation based on practice guidelines referral |
Patients diagnosed with Paget disease of bone should have a radionuclide scan to determine the extent of the disease.16,18,19 | C | Consistent findings from evidence-based practice guidelines |
After initial radiographic diagnosis, patients should be assessed biochemically using total |
References
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