Highlights & Basics
- Paget's disease of bone (PDB) is a chronic localized bone remodeling disorder characterized by increased bone resorption, bone formation, and remodeling, which may lead to major long bone and skull deformities.
- Majority of patients are asymptomatic. Symptomatic patients typically experience pain localized to the bone or joint, either from the pagetic lesion, secondary osteoarthritis, deformity, or pathologic fracture.
- Neurologic symptoms due to bone overgrowth with consequent nerve impingement may include hearing loss, chronic facial pain, hydrocephalus, peripheral nerve entrapment, and spinal stenosis. Very rarely, high-output cardiac failure caused by high blood flow to metabolically active bone sites may occur.
- Diagnosis is incidental in the majority of patients, with an isolated elevated serum alkaline phosphatase raising suspicion for disease. Plain radiographs are diagnostic; in less clear cases, a computed tomography scan is indicated. Bone biopsy is the ultimate confirmatory test, but it is rarely necessary.
- If treatment is indicated, bisphosphonates are the first-line therapy to retard excessive osteoclastic activity. Adjunctive therapy includes physical therapy, orthoses, and walking and hearing aids. Analgesics are indicated for pain control.
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Citations
Tuck SP, Walker J. Adult Paget's disease of bone. Clin Med (Lond). 2020 Nov;20(6):568-71.[Abstract][Full Text]
Singer FR, Bone HG 3rd, Hosking DJ, et al. Paget's disease of bone: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2014 Dec;99(12):4408-22.[Abstract][Full Text]
Whitehouse RW. Paget's disease of bone. Semin Musculoskelet Radiol. 2002 Dec;6(4):313-22.[Abstract]
Ralston SH, Corral-Gudino L, Cooper C, et al. Diagnosis and management of Paget's disease of bone in adults: a clinical guideline. J Bone Miner Res. 2019 Apr;34(4):579-604.[Abstract][Full Text]
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