New guidelines on the diagnosis and management of Paget’s disease of bone (PDB) have been released, providing updated recommendations in areas such as use of bisphosphonates and surgery for the disease.

Developed by the by the Paget’s Association (UK), the guidelines have been endorsed by international expert groups including the European Calcified Tissues Society, the International Osteoporosis Foundation and the American Society of Bone and Mineral Research.

The guidelines published in the Journal of Bone and Mineral Research state that in the diagnosis of Paget’s disease, radionuclide bone scans are recommended in addition to targeted radiographs, as a means of fully and accurately defining the extent of metabolically active disease in patients with PDB.

They also recommend serum total alkaline phosphatase (ALP) as a first‐line biochemical screening test in combination with liver function tests in screening for the presence of metabolically active PDB. However CT and MRI imaging are not recommended for the diagnosis of PDB.

A treatment strategy aimed at improving symptoms is recommended over a treat‐to‐target strategy for increased metabolic activity aimed at normalising total ALP .

Other management recommendations include.

• 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 favourable pain response.
• However bisphosphonates are not recommended to improve quality of life, prevent fractures, progression of osteoarthritis or hearing loss, reduce perioperative blood loss during elective orthopaedic surgery, prevent or treat bone deformity or prevent neoplastic transformation.
• Bisphosphonate therapy may be considered to suppress metabolic activity in PDB, but the clinical benefit is uncertain.
• Measurement of PINP is recommended to predict lesion extent, as defined by scintigraphy, after bisphosphonate therapy.
• A trial of calcitonin treatment may be considered as part of the treatment package in patients who have evidence of neurological dysfunction. Calcitonin may be considered for the short‐term treatment of bone pain where bisphosphonates are contraindicated.
• Denosumab is not recommended but may be considered for the treatment of Giant cell tumour (GCT) of bone when the tumour is nonresectable.
• Measurement of biochemical markers of bone turnover are not recommended a means of predicting the response of bone pain to osteoclast inhibitors in PDB.
• Surgery is recommended for fixation of fractures through affected bone in PDB, but the clinical outcome in femoral neck and subtrochanteric fractures is poor. 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.
• 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.
• Spine surgery may be considered for patients with PDB who develop spinal stenosis and spinal cord compression.

The guideline authors also highlighted the need for more patient‐focused clinical outcomes in PDB and identified areas where further research was needed, such as:

• Risk and benefits of treating asymptomatic patients with PDB
• Effects of treatment on complications
• Role of genetic profiling in the diagnosis of PDB and prediction of complications
• Clinical outcome of joint replacement surgery and osteotomy in the modern era
• Clinical outcome after fracture fixation in the modern era
• Effects of nonpharmacological treatments other than surgery

The guideline authors declared financial interests with several makers of antiresorptive drugs.