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Diagnosis and Management of Paget’s Disease of Bone in Adults: New Guidelines

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Diagnosis and Management of Paget’s Disease of Bone in Adults: New Guidelines
A Guideline Development Group (GDG) led by the Paget’s Association has developed an evidence‐based clinical guideline for the diagnosis and management of Paget’s disease of bone (PDB).

The guideline has 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 and has been published in Journal of Bone and Mineral Research.

Paget’s disease of the bone is a nonmalignant skeletal disorder characterized by focal abnormalities in bone remodeling at one (monostotic) or more (polyostotic) skeletal sites. Almost any bone can be affected, but there is a predilection for the pelvis, spine, femur, tibia, and skull.

The main risk factors for PDB include increasing age, male sex, and ethnic background.The risk of developing PDB increases with age, with an approximate doubling in incidence each decade after the age of 50 years.Paget’s is more common in males (1.4:1)and in certain ethnic groups. Whites are most commonly affected,and the disease has been estimated to affect about 1% of people over the age of 55 years in the United Kingdom.

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Following are the major recommendations:

  1. Plain X‐rays of the abdomen, tibias, skull, and facial bones are recommended as an initial diagnostic screening test in patients suspected to have PDB on biochemical or clinical grounds.
  2. Radionuclide bone scans, in addition to targeted radiographs, are recommended as a means of fully and accurately defining the extent of the metabolically active disease in patients with PDB.
  3. There was insufficient evidence to recommend MRI or CT imaging for the diagnosis of PDB and neither technique is recommended for this purpose. These imaging techniques are recommended for the assessment of disease complications.
  4. Serum total ALP is recommended as a first‐line biochemical screening test in combination with liver function tests in screening for the presence of PDB. If total ALP values are normal and clinical suspicion of metabolically active PDB is high, measurement of BALP, PINP, or uNTX may be considered to screen for metabolically active disease.
  5. Bisphosphonates are recommended for the treatment of bone pain associated with Paget’s disease. Zoledronic acid is recommended as the bisphosphonate most likely to give a favorable pain response.
  6. There is insufficient evidence that bisphosphonate therapy improves quality of life to a clinically meaningful extent in PDB, and they are not recommended for this indication.
  7. There is insufficient evidence that bisphosphonate therapy prevents fractures in PDB, and they are not recommended for this indication.
  8. There is insufficient evidence that bisphosphonate therapy prevents progression of osteoarthritis in PDB, and they are not recommended for this indication.
  9. There is insufficient evidence that bisphosphonate therapy prevents progression of hearing loss in PDB, and they are not recommended for this indication.
  10. There is insufficient evidence that bisphosphonate therapy reduces perioperative blood loss during elective orthopedic surgery, and they are not recommended for this indication.
  11. There is insufficient evidence that bisphosphonates can prevent or treat bone deformity in PDB, and they are not recommended for this indication.
  12. A trial of calcitonin treatment may be considered as part of the treatment package in patients with PDB who have evidence of neurological dysfunction. Bisphosphonate treatment may also be considered, although there are few studies to support the use of bisphosphonates in this situation.
  13. Bisphosphonate therapy may be considered to suppress metabolic activity in PDB, but the clinical benefit is uncertain. Within this class of drugs, nitrogen‐containing bisphosphonates are more effective than non‐nitrogen‐containing bisphosphonates, and within the bisphosphonates, zoledronic acid is most efficacious.
  14. There is insufficient evidence to show that bisphosphonates prevent neoplastic transformation in PDB, and they are not recommended for this indication.
  15. We recommend that patients undergoing treatment with bisphosphonates for PDB are informed about their favorable adverse event profile. We also recommend that patients are advised that a transient flu‐like illness occurs commonly with intravenous zoledronic acid.
  16. Treatment aimed at improving symptoms is recommended over a treat‐to‐target strategy aimed at normalizing total ALP in PDB…
  17. For patients with metabolically active PDB with bone pain treated with neridronate, either the intravenous or intramuscular route can be recommended.
  18. Calcitonin may be considered for the short‐term treatment of bone pain in PDB where bisphosphonates are contraindicated.
  19. Denosumab may be considered for the treatment of GCT complicating PDB when the tumor is nonresectable. There is insufficient evidence to support the use of denosumab in the treatment of PDB, and it is not recommended for this indication.
  20. Measurement of PINP is recommended to predict lesion extent, as defined by scintigraphy, after bisphosphonate therapy.
  21. Measurement of biochemical markers of bone turnover are not recommended a means of predicting the response of bone pain to osteoclast inhibitors in PDB.
  22. Surgery is recommended for fixation of fractures through affected bone in PDB, but the clinical outcome in femoral neck and subtrochanteric fractures is poor. There is insufficient information to recommend one type of surgical treatment over another.
  23. Total hip or knee replacements are recommended for patients with PDB who develop osteoarthritis in whom medical treatment is inadequate. There is insufficient evidence to recommend one type of surgical approach over another for either site.
  24. Osteotomy may be considered for patients with PDB who develop osteoarthritis in whom medical treatment is inadequate, but there is insufficient evidence to make a recommendation on when this technique should be used as opposed to other surgical procedures such as arthroplasty.
  25. Spine surgery may be considered for patients with PDB who develop spinal stenosis and spinal cord compression.
Investigation or indication Recommendation Conditional recommendation Insufficient evidence
Diagnosis of PDB
X‐rays X‐rays of abdomen, skull, facial bone, and tibia recommended
Radionuclide bone scans To fully determine extent of metabolically active disease
MRI and CT Not recommended for diagnosis May be considered to evaluate complications
ALP First‐line biochemical test for metabolically active PDB in combination with LFT
PINP, BALP, NTX Second‐line tests when suspicion of metabolically active disease is high and ALP is normal
Bisphosphonate treatment
Bone pain Recommended for the treatment of bone pain
Quality of life Insufficient evidence; treatment not recommended
Fracture prevention Insufficient evidence; treatment not recommended
Progression of osteoarthritis Insufficient evidence; treatment not recommended
Progression of hearing loss Insufficient evidence; treatment not recommended
Blood loss during elective orthopedic surgery Insufficient evidence; treatment not recommended
Bone deformity Insufficient evidence; treatment not recommended
Neurological symptoms Calcitonin or bisphosphonates may be considered as part of the treatment package
Asymptomatic patients with increased metabolic activity Bisphosphonates may be considered, but clinical benefit unclear
Neoplastic transformation Insufficient evidence; treatment not recommended
Adverse effects of bisphosphonates Patients can be reassured about the favorable adverse event profile
Treatment strategy
Symptomatic or intensive bisphosphonate treatment Treatment goal should be to control bone pain rather than normalize ALP
Route of neridronate administration Intravenous and intramuscular both recommended
Other treatments
Calcitonin for bone pain May be considered for short‐term treatment of bone pain
Denosumab for treatment of PDB Insufficient evidence; treatment not recommended
Denosumab for giant cell tumor May be considered for treatment of giant cell tumor that is unresectable
Predicting response to treatment
Predicting response of bone lesions Measurement of PINP recommended to predict lesion extent defined by scintigraphy after treatment
Predicting response of pain Measurement of biochemical markers is not recommended as a means of predicting response of bone pain
Nonpharmacological treatments
Fracture fixation Surgery is recommended for fixation of fractures through Pagetic bone
Hip or knee arthroplasty Recommended for patients with PDB with OA where medical treatment is inadequate
Osteotomy May be considered for patients with PDB with OA where medical treatment is inadequate

For more details click on the link: https://doi.org/10.1002/jbmr.3657

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