Diagnosis And Management Of Paget's Disease Of Bone In Adults: A Clinical Guideline
S. Ralston, L. Corral-Gudino, C. Cooper, R. Francis, W. Fraser, L. Gennari, N. Guañabens, M. K. Javaid, R. Layfield, T. O'Neill, R. Russell, M. Stone, Keith Simpson, D. Wilkinson, R. Wills, M. Zillikens, S. P. Tuck
Published 2019 · Medicine, Biology
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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 and pharmacological and nonpharmacological treatment options was conducted that 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: 1) Radionuclide bone scans, in addition to targeted radiographs, are recommended as a means of fully and accurately defining the extent of metabolically active disease in patients with PDB. 2) 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. 3) Bisphosphonates are recommended for the treatment of bone pain associated with PDB. Zoledronic acid is recommended as the bisphosphonate most likely to give a favorable pain response. 4) Treatment aimed at improving symptoms is recommended over a treat‐to‐target strategy aimed at normalizing total ALP in PDB. 5) 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. © 2019 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals Inc.
This paper references
Clinical, biochemical and histological observations on the effect of porcine calcitonin in Paget's disease of bone.
T. Martin (1977)
Sarcoma arising in Paget's disease of the skull: case report.
A. Gandolfi (1979)
Skeletal distribution and biochemical parameters of Paget's disease.
P. Meunier (1987)
Relationship between biochemical markers of bone turnover and bone scintigraphic indices in assessment of Paget's disease activity.
L. Alvarez (1997)
Recurrent mutation of the gene encoding sequestosome 1 (SQSTM1/p62) in Paget disease of bone.
N. Laurin (2002)
Total hip arthroplasty in Paget's disease. A follow-up note.
D. J. Mcdonald (1987)
Ultrastructural characteristics of osteoclasts in Paget's disease.
A. Rebel (1974)
[ published erratum appears in Bone 1996 Mar ; 18 ( 3 ) : 292 ]
JY Reginster (1995)
Neurologic disturbances in Paget disease of bone
J. R. Chen (1979)
Paget's disease at the hip with osteoarthrosis: results of intertrochanteric osteotomy.
B. Roper (1971)
Effect of Actinomycin D on Paget's Disease of Bone
J. Fennelly (1971)
A randomized clinical trial comparing oral alendronate and intravenous pamidronate for the treatment of Paget's disease of bone.
J. Walsh (2004)
A multicenter trial of low dose gallium nitrate in patients with advanced Paget's disease of bone.
R. Bockman (1995)
: a review of 889 patients
TP vanStaa (1999)
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein
G. Watts (2004)
Near Complete Necrosis of a Giant Cell Tumor After Treatment with Denosumab: A Case Report.
Elke R. Ahlmann (2012)
Clinical practice. Paget's disease of bone.
S. Ralston (2013)
Canine distemper virus transcripts sequenced from pagetic bone.
M. Gordon (1992)
Paget’s disease of the hip
Javad Parvizi (2004)
Implications of a New Radiological Approach for the Assessment of Paget Disease
N. Guañabens (2012)
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS.
H. J. Kim (2013)
Total knee arthroplasty for osteoarthrosis in patients who have Paget disease of bone at the knee.
G. Gabel (1991)
GRADE Evidence to Decision (EtD) frameworks: a systematic and transparent approach to making well informed healthcare choices. 1: Introduction
P. Alonso-Coello (2016)
Novel UBA Domain Mutations of SQSTM1 in Paget's Disease of Bone: Genotype Phenotype Correlation, Functional Analysis, and Structural Consequences
L. Hocking (2004)
Computed tomography of Paget disease of the skull versus fibrous dysplasia
J. Tehranzadeh (1998)
A comparison of bone scanning and radiology in the assessment of patients with symptomatic Paget's disease
I. Fogelman (2004)
Denosumab in patients with giant-cell tumour of bone: an open-label, phase 2 study.
D. Thomas (2010)
Anti-osteoclastic agent, denosumab, for a giant cell tumor of the bone with concurrent Paget's disease: A case report
Takaaki Tanaka (2017)
Total knee arthroplasty in patients with Paget's disease of bone at the knee.
Gwo-Chin Lee (2005)
Application of GRADE: Making evidence-based recommendations about diagnostic tests in clinical practice guidelines
Jonathan H. Hsu (2011)
Vertebral “clover” scintigraphic image in a vertebral metastasis misdiagnosed with Paget’s disease
R. Reyes (2008)
marrow cultures from patients with Paget ’ s disease of bone
MA Birch (2000)
Measles virus nucleocapsid protein increases osteoblast differentiation in Paget's disease.
J. Teramachi (2016)
Comparison of Different Intravenous Bisphosphonate Regimens for Paget's Disease of Bone
D. Merlotti (2007)
Delayed Development of Paget's Disease in Offspring Inheriting SQSTM1 Mutations
M. Bolland (2007)
Surgical treatment in spine Paget’s disease: a systematic review
Alberto Jorge-Mora (2015)
Comparative study of alendronate versus etidronate for the treatment of Paget's disease of bone.
E. Siris (1996)
The Changing Presentation of Paget’s Disease of Bone in Australia, A High Prevalence Region
Cameron Britton (2017)
Spinal cord dysfunction in Paget's disease of bone. Has medical treatment a vascular basis?
D. Douglas (1981)
PAGET'S DISEASE AT THE HIP WITH OSTEOARTHROSIS: RESULTS OF LNTERTROCHANTERIC OSTEOTOMY
B. Roper (1971)
The Epidemiology of Paget's Disease in Britain: Is the Prevalence Decreasing?
Cyrus Cooper (1999)
Absence of paramyxovirus RNA in cultures of pagetic bone cells and in pagetic bon
M. Birch (1994)
Treatment of Paget’s Disease of Bone with Denosumab: Case Report and Literature Review
I. Reid (2016)
Guidelines on the management of Paget's disease of bone.
P. Selby (2002)
Sarcoma arising in Paget's disease of bone: declining incidence and increasing age at presentation.
D. Mangham (2009)
Evidence for both respiratory syncytial virus and measles virus antigens in the osteoclasts of patients with Paget's disease of bone.
B. Mills (1984)
Epidemiology of Paget's disease of bone
R. Altman (2002)
A randomized, double-blind comparison of risedronate and etidronate in the treatment of paget’s disease of bone
P. Miller (1999)
Epidemiology of Paget's disease of bone: a systematic review and meta-analysis of secular changes.
L. Corral-Gudino (2013)
Secular changes in Paget’s disease: contrasting changes in the number of new referrals and in disease severity in two neighboring regions of Spain
L. Corral-Gudino (2012)
Clinical Presentation of Paget’s Disease: Evaluation of a Contemporary Cohort and Systematic Review
A. Tan (2014)
A single infusion of zoledronic acid produces sustained remissions in paget disease: Data to 6.5 years
I. Reid (2011)
Failure to detect measles virus ribonucleic acid in bone cells from patients with Paget's disease.
B. Matthews (2008)
Paget's disease of bone: a review of 889 patients.
M. Davie (1999)
Paget disease: MR imaging findings.
M. Roberts (1989)
The 'mouse face' appearance of the vertebrae in Paget's disease.
C. Kim (1997)
Paget's disease of bone: review with emphasis on radiologic features, part II
J. Mirra (1995)
JerumsG,MelickRA,Xipell JM,ArnottR.Clinical, biochemical and histological observations on the effect of porcine calcitonin in Paget’s disease of bone
TJ Martin (1977)
Comparison of intravenous and intramuscular neridronate regimens for the treatment of paget disease of bone
D. Merlotti (2011)
Objective tumor response to denosumab in patients with giant cell tumor of bone: a multicenter phase II trial
Takafumi Ueda (2015)
Genome-wide association identifies three new susceptibility loci for Paget's disease of bone
Omar M E Albagha (2011)
Comparison of a single infusion of zoledronic acid with risedronate for Paget's disease.
I. Reid (2005)
Deubiquitinating enzyme CYLD negatively regulates RANK signaling and osteoclastogenesis in mice.
W. Jin (2008)
A randomized clinical trial comparing oral alendronate and intravenous pamidronate for the treatment of Paget ’ s disease of
IR Reid (2004)
Biochemical response to combination of disodium etidronate with calcitonin in Paget's disease.
D. ODonoghue (1987)
Discriminative value of biochemical markers of bone turnover in assessing the activity of Paget's disease
L. Alvarez (1995)
Hearing loss in Paget's disease of bone: evidence of auditory nerve integrity.
E. Monsell (1995)
Paget’s Disease of Bone in New Zealand: Continued Decline in Disease Severity
H. Cundy (2004)
The role of mithramycin in the management of Paget's disease.
D. Heath (1981)
Femoral Fractures in Paget's Disease
S. Bidner (1989)
ZNF 687 mutations in severe Paget disease of bone associatedwith giant cell tumor
Paget's Disease of Bone in The Netherlands: A Population‐Based Radiological and Biochemical Survey—The Rotterdam Study
Marelise E W Eekhoff (2004)
Long‐Term Randomized Trial of Intensive Versus Symptomatic Management in Paget's Disease of Bone: The PRISM‐EZ Study
A. Tan (2017)
Bone scanning in evaluation of Paget's disease of bone.
P. Shirazi (1974)
[GRADE Evidence to Decision (EtD) frameworks: a systematic and transparent approach to making well informed healthcare choices. 2: Clinical practice guidelines.]
G. P. Morgano (2018)
The effect of 1 alpha-hydroxyvitamin D3 on the mineralization defect in disodium etidronate-treated Paget's disease--a double-blind randomized clinical study.
S. Ralston (1987)
Effect of bisphosphonate treatment in patients with Paget's disease of the skull.
J. Donáth (2004)
Tiludronate therapy for Paget's disease of bone.
M. Mcclung (1995)
A randomized, double-blind comparison of risedronate and etidronate in the treatment of Paget's disease of bone. Paget's Risedronate/Etidronate Study Group.
P. Miller (1999)
ZNF687 Mutations in Severe Paget Disease of Bone Associated with Giant Cell Tumor
G. Divisato (2016)
Multicenter Blinded Analysis of RT‐PCR Detection Methods for Paramyxoviruses in Relation to Paget's Disease of Bone
S. Ralston (2007)
Clinical, biochemical, hematologic, and radiographic responses in Paget's disease following intravenous pamidronate disodium: a 2-year study.
D. Gutteridge (1996)
Epidemiological aspects of Paget's disease: family history and relationship to other medical conditions.
E. Siris (1994)
Bone histomorphometry in Paget's disease. Quantitative and dynamic analysis of pagetic and nonpagetic bone tissue.
P. Meunier (1980)
Outcome of Uncemented Hip Arthroplasty Components in Patients With Paget’s Disease
J. Parvizi (2002)
Characterization of and risk factors for the acute-phase response after zoledronic acid.
I. Reid (2010)
Bone turnover markers in Paget’s disease of the bone: A Systematic review and meta-analysis
A. Al Nofal (2015)
The AGREE Reporting Checklist: a tool to improve reporting of clinical practice guidelines
Giant cell tumor in a case of Paget's disease of bone: an aggressive benign tumor exhibiting a quick response to an innovative therapeutic agent. Clin Cases Miner Bone Metab
R Cosso (2010)
A double-blind, multicentre, placebo-controlled study of tiludronate in Paget's disease of bone.
W. Fraser (1997)
Billiard-player's fingers: an unusual case of Paget's disease of bone.
L. Solomon (1979)
Giant cell tumor in a case of Paget's disease of bone: an aggressive benign tumor exhibiting a quick response to an innovative therapeutic agent.
Roberta Cosso (2010)
Skeletal scintimaging and radiography in the diagnosis and management of Paget's disease.
H. Wellman (1977)
Treatment of Paget's disease of bone with porcine calcitonin: clinical and metabolic responses.
J. Kanis (1975)
Biochemical and radiologic improvement in Paget's disease of bone treated with alendronate: a randomized, placebo-controlled trial.
I. Reid (1996)
A family study of Paget's disease of bone.
J. Sofaer (1983)
FRACTURE OF THE FEMUR IN PATIENTS WITH PAGET'S DISEASE; RESULTS OF TREATMENT IN TWENTY-THREE CASES.
J. Nicholas (1965)
Safety and efficacy of denosumab for adults and skeletally mature adolescents with giant cell tumour of bone: interim analysis of an open-label, parallel-group, phase 2 study.
S. Chawla (2013)
Genetics and aetiology of Pagetic disorders of bone.
M. Helfrich (2008)
Evaluation of the efficacy and safety of oral tiludronate in Paget's disease of bone. A double-blind, multiple-dosage, placebo-controlled study.
J. Reginster (1992)
The clover and heart signs in vertebral scintigraphic images are highly specific of Paget's disease of bone.
D. Rotés-Sala (2004)
Adult Paget's disease of bone: a review.
Stephen Paul Tuck (2017)
Clinical Determinants of Quality of Life in Paget’s Disease of Bone
A. Langston (2006)
Detection of measles virus nucleocapsid transcripts in circulating blood cells from patients with paget disease
S. Reddy (1996)
Familial Aggregation of Paget's Disease of Bone
E. Siris (1991)
Paget's disease of bone: an endocrine society clinical practice guideline.
F. Singer (2014)
Paget's disease in New Zealand: evidence for declining prevalence.
T. Doyle (2002)
Observations on serial radionuclide blood-flow studies in Paget's disease: concise communication.
R. Boudreau (1983)
The management of fractures in Paget's disease of bone.
D. Verinder (1979)
Mutations in TNFRSF11A, affecting the signal peptide of RANK, cause familial expansile osteolysis
A. Hughes (2000)
in Lancashire and arsenic pesticide in cottonmill wastewater : a speculative hypothesis
LR Solomon (2002)
Bisphosphonates for Paget's disease of bone in adults.
L. Corral-Gudino (2017)
Sequestosome 1: Mutation Frequencies, Haplotypes, and Phenotypes in Familial Paget's Disease of Bone
J. Morissette (2006)
Improvement in the deformity of the face in Paget's disease treated with diphosphonates.
D. Bickerstaff (1990)
Outcome after fractures of the femur in Paget's disease.
C. M. Bradley (1992)
Evaluation of the Role of the SQSTM1 Gene in Sporadic Belgian Patients with Paget’s Disease
G. Beyens (2004)
The effect of 1α‐hydroxyvitamin D3 on the mineralization defect in disodium etidronate‐treated paget's disease — a double‐blind randomized clinical study
S. Ralston (1987)
Failure to detect paramyxovirus sequences in paget's disease of bone using the polymerase chain reaction
S. Ralston (1991)
Incidence and Natural History of Paget's Disease of Bone in England and Wales
T. V. van Staa (2002)
Archaeological skeletons support a northwest European origin for Paget's disease of bone
S. Mays (2010)
Clinical Characteristics and Evolution of Giant Cell Tumor Occurring in Paget's Disease of Bone
D. Rendina (2015)
Guidelines for diagnosis and management of Paget's disease of bone in Japan
S. Takata (2006)
p62 at the Crossroads of Autophagy, Apoptosis, and Cancer
J. Moscat (2009)
Corrective Osteotomy for Deformity in Paget Disease
J. Parvizi (2003)
Giant Cell Tumor Developing in Paget’s Disease of Bone: A Case Report with Review of Literature
Vivek Verma (2016)
Domain-specific mutations in sequestosome 1 (SQSTM1) cause familial and sporadic Paget's disease.
L. Hocking (2002)
Osteosarcoma incidence and survival rates from 1973 to 2004
L. Mirabello (2009)
Osteoprotegerin deficiency and juvenile Paget's disease.
L. Hofbauer (2002)
Papilloedema in Guillain-Barré syndrome.
T. Perniola (1968)
Familial Paget's disease of bone: patterns of inheritance and frequency of linkage to chromosome 18q.
L. Hocking (2000)
Diphosphonate therapy of paget's disease of bone.
R. Canfield (1977)
Cementless total hip arthroplasty in Paget’s disease of bone: a retrospective review
J. Wegrzyn (2009)
PAST PETS AND PAGET'S DISEASE
J. O'driscoll (1985)
Trauma and Paget's disease of bone.
R. Hamdy (1979)
Antibody Response to Paramyxoviruses in Paget’s Disease of Bone
Micaela Rios Visconti (2017)
Grading quality of evidence and strength of recommendations
D. Atkins (2004)
Fractures of the femur in Paget's disease of bone. Their etiology and treatment.
M. Grundy (1970)
Frequency and characteristics of familial aggregation of paget's disease of bone
A. Morales-Piga (1995)
Glucagon in the Treatment of Paget's Disease of Bone
J. Condon (1971)
Paget's disease of bone in Lancashire and arsenic pesticide in cotton mill wastewater: a speculative hypothesis.
J. H. Lever (2002)
Influence of disodium etidronate on clinical and laboratory manifestations of Paget's disease of bone (osteitis deformans).
R. D. Altman (1973)
Absence of measles virus and canine distemper virus transcripts in long-term bone marrow cultures from patients with Paget's disease of bone.
C. Ooi (2000)
The Role of Surgery and Adjuvants to Survival in Pagetic Osteosarcoma
P. Ruggieri (2010)
Novel Serum Markers of Bone Resorption: Clinical Assessment and Comparison with Established Urinary Indices
H. Woitge (1999)
Randomized trial of intensive bisphosphonate treatment versus symptomatic management in paget's disease of bone
A. Langston (2010)
A Negative Search for a Paramyxoviral Etiology of Paget's Disease of Bone: Molecular, Immunological, and Ultrastructural Studies in U.K. Patients
M. Helfrich (2000)
Epidemiology of Paget's Disease in Europe: The Prevalence Is Decreasing
G. Poór (2006)
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The loss of Profilin 1 causes early-onset Paget's disease of bone.
Federica Scotto di Carlo (2020)
Tartrate-resistant acid phosphatase 5b, but not periostin, is useful for assessing Paget's disease of bone.
Núria Guañabens (2019)
Paget’s Disease and Secondary Hyperparathyroidism: Is Healing Possible?
Vincenzo Antonio Panuccio (2020)
Management of Paget’s disease of bone
I R Reid (2019)
Recent advances in understanding and managing Paget’s disease
Ian R. Reid (2019)
Paget's Disease of Bone Affecting Peripheral Limb: Difficulties in Diagnosis: A Case Report
Jun-Ku Lee (2020)
Bisphosphonates in the management of Paget's disease.
Stuart H Ralston (2020)
Proceedings of the 2019 Santa Fe Bone Symposium: New Concepts in the Care of Osteoporosis and Rare Bone Diseases.
E Michael Lewiecki (2019)
Management of hydrocephalus in Paget's disease of bone: systematic review and illustrative case.
Abdelsimar T Omar (2019)
Bisphosphonates and atrial fibrillation: revisiting the controversy.
Ibrahim T. Fazmin (2020)
Paget's disease of bone in two medieval skeletons from Poulton Chapel, Cheshire, UK
Carla L. Burrell (2019)
Zoledronate in the prevention of Paget’s (ZiPP): protocol for a randomised trial of genetic testing and targeted zoledronic acid therapy to prevent SQSTM1-mediated Paget’s disease of bone
O. Cronin (2019)
Reply to Clinical Guidelines on Paget's Disease
B. Langdahl (2019)
A Clinical Guideline on Paget’s Disease of bone—A Guideline or a Review?
B. Langdahl (2019)
Quantitative Bone Single Photon Emission Computed Tomography/Computed Tomography for Evaluating Response to Bisphosphonate Treatment in Patients with Paget's Disease of Bone
K. Kitajima (2020)
Characteristics of Early Paget's Disease in SQSTM1 Mutation Carriers: Baseline Analysis of the ZiPP Study Cohort
O. Cronin (2020)
Clinical features, diagnosis and treatment of Paget’s disease of bone in mainland China: A systematic review
Q. Wang (2020)
Familial Paget's disease of bone: Long-term follow-up of unaffected relatives with and without Sequestosome 1 mutations.
J. J. M. Peeters (2019)
Focal and Osteosclerotic Bone Diseases
S. Ralston (2019)
Clinical Guidelines on Paget's Disease of Bone
S. Ralston (2019)