Diagnosis and Management of Paget's Disease of Bone in Adults: A Clinical Guideline

Abstract

An evidence based clinical guideline for the diagnosis and management of Paget’s disease of Bone (PDB) was developed using GRADE methodology, by a Guideline Development Group (GDG) led by the Paget’s Association (UK). A systematic review of diagnostic tests, pharmacological and non-pharmacological treatment options was conducted which sought to address several key questions of clinical relevance. Twelve recommendations and five conditional recommendations were made but there was insufficient evidence to address eight of the questions posed. The following recommendations were identified as the most important. 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.Serum total alkaline phosphatase (ALP) is recommended as a first line biochemical screening test in combination with liver function tests in screening for the presence of metabolically active PDB.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.Treatment aimed at improving symptoms is recommended over a treat-to-target strategy aimed at normalising total ALP in PDB.Total hip or knee replacements are recommended for patients with PDB who develop osteoarthritis in whom medical treatment is inadequate. There is insufficient information to recommend one type of surgical approach over another. The guideline was 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. The GDG noted that there had been a lack of research on patient-focused clinical outcomes in PDB and identified several areas where further research was needed.

Forearm lump and osteoporosis

“…Bone deformities as it might have been in this case are identified especially in pressure areas (8,9,10). Serum total alkaline phosphatase remains the first line of assays even less than on fifths of cases had normal values (5,10,11). In this case the bone turnover were within normal limits except for a small decrease of CrossLaps.…”

“…It has a relative high frequency in some populations but less than primary osteoporosis (7). Bone deformities as it might have been in this case are identified especially in pressure areas (8,9,10). Serum total alkaline phosphatase remains the first line of assays even less than on fifths of cases had normal values (5,10,11).…”

“…Radionuclide bone scans are useful for positive diagnosis and for differential diagnosis as seen here regarding bone metabolic conditions (5,10). Even a prior undetected fracture was identified at the rib the region with deformation was not active.…”

“…A recent fracture increases the bone markers for a while and they are not useful for establishing the diagnosis of osteoporosis (12). In Paget's disease or multiple myeloma the markers may reach to extremely high values (10,12). Under biphosphonates the markers get low opposite to teriparatide therapy as an osteoanabolic option (13,14,15).…”

Clinical Guidelines on Paget's Disease of Bone

“…As authors of the recently published clinical guideline on the diagnosis and management of Paget's disease we were delighted to see that our article was thought by the Editors of JBMR to be of sufficient interest to warrant an editorial . However, we were astonished to read in the editorial that “the new PDB guideline is a review more than a clinically relevant guideline” apparently because we did not make recommendations where there was a lack of evidence, unlike the 2014 Endocrine Society Guideline …”