Skip to main content
Top
Published in: Rheumatology International 11/2008

01-09-2008 | Review

Paget’s disease of bone: a review

Authors: Matteo Colina, Renato La Corte, Francesco De Leonardis, Francesco Trotta

Published in: Rheumatology International | Issue 11/2008

Login to get access

Abstract

Paget’s disease of bone (PDB) is a condition of unknown etiology characterized by excessive and abnormal bone remodeling. It may be localized to one or several skeletal segments. The disease seldom appears before the age of 40 years, but its prevalence tends to double each decade from the age of 50 onwards, reaching about 10% after ninth decade. PDB may virtually affect every bone in the skeleton. Affected bones are involved right away with no new involvement during the evolution. The basic symptom of the disease is bone pain, while complications depend on skeletal sites involved and range from secondary osteoarthritis to malignant degeneration. Diagnosis is usually based upon clinical features, imaging, and laboratory analyses. Therapeutic approach is currently based on second-generation bisphosphonates. Their use is recommended when bone alkaline phosphatase is high and/or when the disease localizations are highly suspected for determining complications.
Literature
1.
go back to reference Nagant de Deuxchaisnes C, Krane SM (1964) Paget’s disease of bone. Clinical and metabolic observations. Medicine 43:233–266 Nagant de Deuxchaisnes C, Krane SM (1964) Paget’s disease of bone. Clinical and metabolic observations. Medicine 43:233–266
2.
go back to reference Roberts C, Manchester K (1995) The archeology of disease. Sutton Publishing, Phoenix Mill Roberts C, Manchester K (1995) The archeology of disease. Sutton Publishing, Phoenix Mill
3.
go back to reference Barker DJ, Clough PW, Guyer PB, Gardner MJ (1977) Paget’s disease of bone in 14 British towns. BMJ 1:1181–1183PubMed Barker DJ, Clough PW, Guyer PB, Gardner MJ (1977) Paget’s disease of bone in 14 British towns. BMJ 1:1181–1183PubMed
4.
go back to reference Barker DJ, Chamberlain AT, Guyer PB, Gardner MJ (1980) Paget’s disease of bone: the Lancashire focus. BMJ 280:1105–1107PubMed Barker DJ, Chamberlain AT, Guyer PB, Gardner MJ (1980) Paget’s disease of bone: the Lancashire focus. BMJ 280:1105–1107PubMed
5.
go back to reference Gardner MJ, Guyer PB, Barker DJ (1978) Radiological prevalence of Paget’s disease of bone in British migrants to Australia. BMJ 1:1665–1657 Gardner MJ, Guyer PB, Barker DJ (1978) Radiological prevalence of Paget’s disease of bone in British migrants to Australia. BMJ 1:1665–1657
8.
go back to reference Detheridge FM, Guyer PB, Barker DJP (1982) European distribution of Paget’s disease of bone. Br Med J (Clin Res Ed) 285:1005–1008 Detheridge FM, Guyer PB, Barker DJP (1982) European distribution of Paget’s disease of bone. Br Med J (Clin Res Ed) 285:1005–1008
9.
go back to reference Bhadada S, Bhansali A, Unnikrishnan AG, Khadgawat R, Singh SK, Mithal A et al (2006) Does Paget’s disease of bone exist in India? A report of 21 patients. J Assoc Physicians India 54:530–534PubMed Bhadada S, Bhansali A, Unnikrishnan AG, Khadgawat R, Singh SK, Mithal A et al (2006) Does Paget’s disease of bone exist in India? A report of 21 patients. J Assoc Physicians India 54:530–534PubMed
14.
go back to reference Kanis JA (1988) Pathophysiology and treatment of Paget’s disease of bone, 2nd edn. Martin Dunitz, London Kanis JA (1988) Pathophysiology and treatment of Paget’s disease of bone, 2nd edn. Martin Dunitz, London
15.
go back to reference Morales-Piga AA, Rey-Rey JS, Corres-Gonzalez J, Garcia-Sagredo JM, Lopez-Abente G (1995) Frequency and characteristics of familial aggregation of Paget’s disease of bone. J Bone Miner Res 10:663–670PubMed Morales-Piga AA, Rey-Rey JS, Corres-Gonzalez J, Garcia-Sagredo JM, Lopez-Abente G (1995) Frequency and characteristics of familial aggregation of Paget’s disease of bone. J Bone Miner Res 10:663–670PubMed
16.
go back to reference Sofaer JA, Holloway SM, Emery AE (1983) A family study of Paget’s disease of bone. J Epidemiol Community Health 37:226–231PubMedCrossRef Sofaer JA, Holloway SM, Emery AE (1983) A family study of Paget’s disease of bone. J Epidemiol Community Health 37:226–231PubMedCrossRef
21.
go back to reference Singer FR (1999) Update on the viral etiology of Paget’s disease of bone. J Bone Miner Res 14:S29–S33 Singer FR (1999) Update on the viral etiology of Paget’s disease of bone. J Bone Miner Res 14:S29–S33
22.
go back to reference Reddy SV, Singer FR, Roodman GD (1995) Bone marrow mononuclear cells from patients with Paget’s disease contain measles virus nucleocapsid messenger ribonucleic acid that has mutations in a specific region of the sequence. J Clin Endocrinol Metab 80:2108–2111. doi:10.1210/jc.80.7.2108 PubMedCrossRef Reddy SV, Singer FR, Roodman GD (1995) Bone marrow mononuclear cells from patients with Paget’s disease contain measles virus nucleocapsid messenger ribonucleic acid that has mutations in a specific region of the sequence. J Clin Endocrinol Metab 80:2108–2111. doi:10.​1210/​jc.​80.​7.​2108 PubMedCrossRef
24.
25.
go back to reference Lucas GJA, Daroszewska A, Ralston SH (2007) Contribution of genetic factors to the pathogenesis of Paget’s disease of bone and related disorders. J Bone Miner Res 21:31–P37. doi:10.1359/jbmr.06s206 CrossRef Lucas GJA, Daroszewska A, Ralston SH (2007) Contribution of genetic factors to the pathogenesis of Paget’s disease of bone and related disorders. J Bone Miner Res 21:31–P37. doi:10.​1359/​jbmr.​06s206 CrossRef
26.
go back to reference Hughes AE, Ralston SH, Marken J, Bell C, MacPherson H, Wallace RG et al (2000) Mutations in TNFRSF11A, affecting the signal peptide of RANK, cause familial expansile osteolysis. Nat Genet 24:45–48. doi:10.1038/71667 PubMedCrossRef Hughes AE, Ralston SH, Marken J, Bell C, MacPherson H, Wallace RG et al (2000) Mutations in TNFRSF11A, affecting the signal peptide of RANK, cause familial expansile osteolysis. Nat Genet 24:45–48. doi:10.​1038/​71667 PubMedCrossRef
27.
go back to reference Meunier PJ, Coindre JM, Edouard CM, Arlot ME (1980) Bone histomorphometry in Paget’s disease. Quantitative and dynamic analysis of pagetic and nonpagetic bone tissues. Arthritis Rheum 23:1095–1103. doi:10.1002/art.1780231005 PubMedCrossRef Meunier PJ, Coindre JM, Edouard CM, Arlot ME (1980) Bone histomorphometry in Paget’s disease. Quantitative and dynamic analysis of pagetic and nonpagetic bone tissues. Arthritis Rheum 23:1095–1103. doi:10.​1002/​art.​1780231005 PubMedCrossRef
31.
go back to reference Maldague B, Malghem J (1989) Medical imaging of Paget’s disease. Rev Prat 39:1113–1124PubMed Maldague B, Malghem J (1989) Medical imaging of Paget’s disease. Rev Prat 39:1113–1124PubMed
32.
go back to reference Mirra JM, Brien EW, Tehranzadeh J (1995) Paget’s disease of bone: review with emphasis on radiologic features, part I. Skeletal Radiol 24:163–171PubMed Mirra JM, Brien EW, Tehranzadeh J (1995) Paget’s disease of bone: review with emphasis on radiologic features, part I. Skeletal Radiol 24:163–171PubMed
36.
go back to reference Smith SE, Murphey MD, Motamedi K, Mulligan ME, Resnick CS, Gannon FH (2002) From the archives of the AFIP. Radiologic spectrum of Paget’s disease of bone and its complications with pathologic correlation. Radiographics 22:1191–1216PubMed Smith SE, Murphey MD, Motamedi K, Mulligan ME, Resnick CS, Gannon FH (2002) From the archives of the AFIP. Radiologic spectrum of Paget’s disease of bone and its complications with pathologic correlation. Radiographics 22:1191–1216PubMed
37.
go back to reference Moser RP Jr, Vinh TN, Ros PR, Smirniotopoulos JG, Madewell JE, Berrey BH (1987) Paget disease of the anterior tibial tubercle. Radiology 164:211–214PubMed Moser RP Jr, Vinh TN, Ros PR, Smirniotopoulos JG, Madewell JE, Berrey BH (1987) Paget disease of the anterior tibial tubercle. Radiology 164:211–214PubMed
39.
go back to reference Papapoulos SE, Frolich M, Mudde AH, Harinck HIJ, Berg HVD, Bijvoet OLM (1987) Serum osteocalcin in Paget’s disease of bone: basal concentrations and response to bisphosphonate treatment. J Clin Endocrinol Metab 65:89–94PubMed Papapoulos SE, Frolich M, Mudde AH, Harinck HIJ, Berg HVD, Bijvoet OLM (1987) Serum osteocalcin in Paget’s disease of bone: basal concentrations and response to bisphosphonate treatment. J Clin Endocrinol Metab 65:89–94PubMed
40.
go back to reference Alvarez L, Peris P, Pons F, Guanabens N, Herranz R, Monegal A et al (1997) Relationship between biochemical markers of bone turnover and bone scintigraphic indices in assessment of Paget’s disease activity. Arthritis Rheum 40:461–468. doi:10.1002/art.1780400312 PubMedCrossRef Alvarez L, Peris P, Pons F, Guanabens N, Herranz R, Monegal A et al (1997) Relationship between biochemical markers of bone turnover and bone scintigraphic indices in assessment of Paget’s disease activity. Arthritis Rheum 40:461–468. doi:10.​1002/​art.​1780400312 PubMedCrossRef
41.
42.
go back to reference Alvarez L, Guanabens N, Peris P, Monegal A, Bedini JL, Deulofeu R et al (1997) Discriminating value of biochemical markers of bone turnover in assessing the activity of Paget’s disease. J Bone Miner Res 10:458–465CrossRef Alvarez L, Guanabens N, Peris P, Monegal A, Bedini JL, Deulofeu R et al (1997) Discriminating value of biochemical markers of bone turnover in assessing the activity of Paget’s disease. J Bone Miner Res 10:458–465CrossRef
45.
go back to reference Siris ES, Weinstein RS, Altman R, Conte JM, Favus M, Lombardi A et al (1996) Comparative study of alendronate vs etidronate for the treatment of Paget’s disease of bone. J Clin Endocrinol Metab 81:961–967. doi:10.1210/jc.81.3.961 PubMedCrossRef Siris ES, Weinstein RS, Altman R, Conte JM, Favus M, Lombardi A et al (1996) Comparative study of alendronate vs etidronate for the treatment of Paget’s disease of bone. J Clin Endocrinol Metab 81:961–967. doi:10.​1210/​jc.​81.​3.​961 PubMedCrossRef
46.
go back to reference Reid I, Nicholson GC, Weinstein RS, Hosking DJ, Cundy T, Kotowicz MA et al (1996) Biochemical and radiologic improvement in Paget’s disease of bone treated with alendronate. A randomized placebo-controlled trial. Am J Med 101:341–348. doi:10.1016/S0002-9343(96)00227-6 PubMedCrossRef Reid I, Nicholson GC, Weinstein RS, Hosking DJ, Cundy T, Kotowicz MA et al (1996) Biochemical and radiologic improvement in Paget’s disease of bone treated with alendronate. A randomized placebo-controlled trial. Am J Med 101:341–348. doi:10.​1016/​S0002-9343(96)00227-6 PubMedCrossRef
47.
go back to reference Miller PD, Brown JP, Siris ES, Hoseyni MS, Axelrod DW, Bekker PJ (1999) A prospective, multicenter, randomized, double-blind comparison of risedronate and etidronate in the treatment of Paget’s disease of bone. Am J Med 106:513–520. doi:10.1016/S0002-9343(99)00062-5 PubMedCrossRef Miller PD, Brown JP, Siris ES, Hoseyni MS, Axelrod DW, Bekker PJ (1999) A prospective, multicenter, randomized, double-blind comparison of risedronate and etidronate in the treatment of Paget’s disease of bone. Am J Med 106:513–520. doi:10.​1016/​S0002-9343(99)00062-5 PubMedCrossRef
48.
go back to reference Trombetti A, Arlot M, Thevenon J, Uebelhart B, Meunier PJ (1999) Effects of multiple intravenous pamidronate courses in Paget’s disease of bone. Rev Rhum Engl Ed 66:467–476PubMed Trombetti A, Arlot M, Thevenon J, Uebelhart B, Meunier PJ (1999) Effects of multiple intravenous pamidronate courses in Paget’s disease of bone. Rev Rhum Engl Ed 66:467–476PubMed
49.
go back to reference Reid IR, Miller P, Lyles K, Fraser W, Brown J, Saidi Y et al (2005) Comparison of a single infusion of zoledronic acid with risedronate for Paget’s disease. N Engl J Med 353:898–908. doi:10.1056/NEJMoa044241 PubMedCrossRef Reid IR, Miller P, Lyles K, Fraser W, Brown J, Saidi Y et al (2005) Comparison of a single infusion of zoledronic acid with risedronate for Paget’s disease. N Engl J Med 353:898–908. doi:10.​1056/​NEJMoa044241 PubMedCrossRef
Metadata
Title
Paget’s disease of bone: a review
Authors
Matteo Colina
Renato La Corte
Francesco De Leonardis
Francesco Trotta
Publication date
01-09-2008
Publisher
Springer-Verlag
Published in
Rheumatology International / Issue 11/2008
Print ISSN: 0172-8172
Electronic ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-008-0640-6

Other articles of this Issue 11/2008

Rheumatology International 11/2008 Go to the issue