Paget's Disease of Bone
Publication Date: December 1, 2014
Last Updated: September 2, 2022
Diagnosis
Imaging
In patients with suspected Paget’s disease, the Endocrine Society (ES) recommends obtaining plain radiographs of the suspicious regions of the skeleton. ( 1 , H )
700
In patients diagnosed with Paget’s disease, the ES suggests a radionuclide bone scan to determine the extent of the disease and identify possible asymptomatic sites. ( 2 , M )
700
Biochemistry
The ES recommends that after radiological diagnosis of Paget’s disease, the initial biochemical evaluation of a patient should be done using serum total alkaline phosphatase (ALP) or with the use of a more specific marker of bone formation when appropriate. ( 1 , H )
700
Treatment
Indications
The ES recommends treatment with a bisphosphonate (see Table 2) for most patients with active Paget’s disease who are at risk of future complications. ( 1 , M )
700
Choice of Medication
The ES suggests a single 5-mg dose of IV zoledronate as the treatment of choice in patients without contraindications. (2, M)
700
Assessing the Response to Treatment
If there is urgency in the control of symptoms or the disease is particularly active, the ES suggests the use of short-term response of bone resorption markers before and shortly after treatment to indicate that an adequate therapeutic response is likely. ( 2 , L )
700
The ES suggests that patients who have osteolytic lesions of Paget’s disease have a repeat x-ray approximately 1 year after radiological diagnosis to determine whether there has been improvement with therapy or worsening in the absence of therapy. Subsequent x-rays may be considered in the event of persistent elevations of biochemical markers of bone turnover or the presence of bone pain and to determine when there is resolution of the lesion. ( 2 , L )
700
Maintaining Remission
The ES suggests that to maximize the duration of remission, bone turnover should be reduced below the midpoint of the reference range for the chosen monitoring bone turnover marker. ( 2 , L )
700
Relapse and Retreatment
The ES recommends that in patients with increased bone turnover, biochemical follow-up should be used as a more objective indicator of relapse than symptoms. ( 1 , M )
700
Monostotic Paget’s Disease
The ES suggests that amino-terminal propeptide of type 1 collagen (P1NP) or bone-specific ALP (BSAP) and βC-terminal propeptide of type 1 collagen (βCTx) or N-terminal propeptide of type 1 collagen (NTx) should be used for assessing the activity of untreated monostotic Paget’s disease, although these may be normal when evidence of disease activity is still clearly demonstrated on scintigraphy. ( 2 , L )
700
Management of the Complications of Paget’s Disease
Hearing Loss
The ES suggests treatment with a potent bisphosphonate to prevent worsening of a hearing deficit. ( 2 , L )
700
Osteoarthritis
The ES suggests the use of analgesics as adjunctive therapy for mild-to-moderate joint pain due to joint cartilage deterioration in patients with Paget’s disease adjacent to the painful joint. ( 2 , L )
700
For patients with severe osteoarthritis adjacent to Paget’s disease of bone, the ES suggests bisphosphonate therapy before undergoing elective total joint replacement to prevent intraoperative hemorrhaging and postoperative loosening of the prosthesis. ( 2 , L )
700
Bowing of Lower Extremity
The ES suggests treatment with a potent bisphosphonate before elective surgery for patients who require an osteotomy to correct severe bowing of the lower extremity associated with impaired ambulation and/or severe joint pain. ( 2 , L )
700
Paralysis
In cases of paraplegia associated with Paget’s disease of the spine, the ES suggests immediate treatment with a potent IV bisphosphonate associated with neurosurgical consultation. Surgical intervention may not be necessary after effective medical treatment unless there is severe structural damage. ( 2 , L )
700
Neoplasms
The ES suggests that patients with osteosarcoma or a giant cell tumor be evaluated by an orthopedic surgeon (ungraded recommendation). If surgery is planned, the ES suggests pretreatment with a potent bisphosphonate to reduce bleeding from adjacent pagetic bone. ( 2 , L )
700
Congestive Heart Failure
The ES suggests treatment with a bisphosphonate in patients with Paget’s disease and congestive heart failure. ( 2 , L )
700
Recommendation Grading
Overview
Title
Paget's Disease of Bone
Authoring Organization
Endocrine Society
Publication Month/Year
December 1, 2014
Last Updated Month/Year
April 3, 2024
Supplemental Implementation Tools
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Document Objectives
The aim of this guideline was to formulate practice guidelines for the diagnosis and treatment of Paget's disease of the bone.
Inclusion Criteria
Male, Female, Adult, Older adult
Health Care Settings
Ambulatory
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Diagnosis, Treatment, Management
Diseases/Conditions (MeSH)
D010144 - Paget's Disease, Mammary, D010145 - Paget Disease, Extramammary
Keywords
Paget's disease of bone, bone disorders, paget's disease
Source Citation
Frederick R. Singer, Henry G. Bone, III, David J. Hosking, Kenneth W. Lyles, Mohammad Hassan Murad, Ian R. Reid, Ethel S. Siris, Paget's Disease of Bone: An Endocrine Society Clinical Practice Guideline, The Journal of Clinical Endocrinology & Metabolism, Volume 99, Issue 12, 1 December 2014, Pages 4408–4422, https://doi.org/10.1210/jc.2014-2910