Paget Disease Guidelines

Updated: Dec 08, 2020
  • Author: Mujahed M Alikhan, MD; Chief Editor: Herbert S Diamond, MD  more...
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Guidelines

Guidelines Summary

The following organizations have issued guidelines on the diagnosis and management of Paget disease of bone (PDB):

  • Endocrine Society 
  • Paget's Association (UK) 

The guidelines from the Guideline Development Group (GDG) of the Paget's Association (United Kingdom), published in 2019, have been endorsed by the European Calcified Tissues Society, the International Osteoporosis Foundation, the American Society of Bone and Mineral Research, the Bone Research Society (UK), and the British Geriatric Society. [54]

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Paget's Association

The key recommendations of the Paget's Association guidelines include the following [54] :

  • Imaging :  X‐rays of abdomen, skull, facial bone for initial diagnostic screening in patients with clinical or biochemical indications of PDB. Radionuclide bone scans are a second-line modality to determine the extent of metabolically active disease. MRI and CT are not recommended for the diagnosis of PDB but are recommended to assess complications.
  • Biomarkers:  Serum total alkaline phosphatase (ALP) in combination with liver function tests is the first‐line biochemical screening test for PDB. In clinically suspicious PDB, if total ALP values are normal, second-line tests include measurement of bone-specific alkaline phosphatase (BALP), N-terminal propeptide of human procollagen type I (PINP), or urinary N-telopeptide (uNTx).
  • Bisphosphonate​ therapy:  Bisphosphonates are the recommended treatment for bone pain associated with Paget disease. It may also be considered for asymptomatic patients with increased metabolic activity. There is insufficient evidence for the following indications: improvement in quality of life, prevention of fractures and bone deformity, prevention of progression of osteoarthritis and hearing loss, and reduction of blood loss prior to elective orthopedic surgery.
  • Treatment of neurologic symptoms:   Calcitonin or bisphosphonate therapy may be considered.
  • Surgical treatment: Spinal surgery should be considered for treatment of spinal stenosis or cord compression. Surgery is recommended for fixation of fractures. Joint replacement surgery and osteotomy are recommended for treatment-resistant osteoarthritis.

The guidelines also pointed to the following unresolved questions in Paget disease management that require further investigation [54] :

  • Treatment of asymptomatic patients
  • Effect of treatment on complications
  • Use of genetic profiling in diagnosis and treatment
  • Benefits of joint replacement surgery, osteotomy, and fracture fixation
  • Potential of less-invasive nonpharmacologic therapies 
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Endocrine Society Guidelines

A 2014 Endocrine Society clinical practice guideline on Paget disease includes the following recommendations [55] :

  • Obtain plain radiographs of the pertinent regions of the skeleton in patients with suspected Paget disease.
  • After diagnosis of Paget disease, measure serum total alkaline phosphatase or, when warranted, a more specific marker of bone formation or bone resorption to assess the response to treatment or the evolution of the disease in untreated patients.

In addition, the Endocrine Society guideline contains the following suggestions [55] :

  • Obtain a radionucleotide bone scan to determine the extent of the disease if the diagnosis is confirmed.
  • Prescribe treatment with a bisphosphonate for most patients with active Paget disease who are at risk for future complications.
  • Administer a single 5-mg dose of intravenous zoledronate as the treatment of choice in patients who have no contraindication.
  • In patients with monostotic disease who have a normal serum total alkaline phosphatase concentration, measure a specific marker of bone formation and bone resorption.
  • If the markers are normal, consider serial radionuclide bone scans to determine the response to treatment.
  • Bisphosphonate treatment may be effective in preventing or slowing the progress of hearing loss and osteoarthritis in joints adjacent to Paget disease and may reverse paraplegia associated with spinal Paget disease.
  • Consider treatment with a bisphosphonate before surgery on pagetic bone.
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