PDB is the second most common chronic bone-remodelling disorder after osteoporosis.[3]Tuck SP, Walker J. Adult Paget's disease of bone. Clin Med (Lond). 2020 Nov;20(6):568-71.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7687326
http://www.ncbi.nlm.nih.gov/pubmed/33199322?tool=bestpractice.com
The majority of cases are sporadic, but between 5% and 40% of patients report a first-degree relative with PDB.[4]Nebot Valenzuela E, Pietschmann P. Epidemiology and pathology of Paget's disease of bone: a review. Wien Med Wochenschr. 2017 Feb;167(1-2):2-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266784
http://www.ncbi.nlm.nih.gov/pubmed/27600564?tool=bestpractice.com
An overall prevalence of 2% has been reported in the UK.[5]Cooper C, Schaftheutle K, Dennison E, et al. The epidemiology of Paget's disease in Britain: is the prevalence decreasing? J Bone Miner Res. 1999 Feb;14(2):192-7.
https://asbmr.onlinelibrary.wiley.com/doi/full/10.1359/jbmr.1999.14.2.192
http://www.ncbi.nlm.nih.gov/pubmed/9933472?tool=bestpractice.com
Prevalence in Europe is 0.3%.[6]Poor G, Donath J, Fornet B, et al. Epidemiology of Paget's disease in Europe: the prevalence is decreasing. J Bone Mineral Res. 2006 Oct;21(10):1545-9.
https://asbmr.onlinelibrary.wiley.com/doi/full/10.1359/jbmr.060704
http://www.ncbi.nlm.nih.gov/pubmed/16995808?tool=bestpractice.com
In the US, prevalence is approximately 1% in the general population, rising to 2.32% in people aged 65 to 74 years.[7]Altman RD, Bloch DA, Hochberg MC, et al. Prevalence of pelvic Paget's disease of bone in the United States. J Bone Miner Res. 2000 Mar;15(3):461-5.
http://www.ncbi.nlm.nih.gov/pubmed/10750560?tool=bestpractice.com
The mean age of onset is 55 years, with equal distribution among men and women (although there is a slight male predominance in the 45- to 74-years age group).[7]Altman RD, Bloch DA, Hochberg MC, et al. Prevalence of pelvic Paget's disease of bone in the United States. J Bone Miner Res. 2000 Mar;15(3):461-5.
http://www.ncbi.nlm.nih.gov/pubmed/10750560?tool=bestpractice.com
A decrease in prevalence since the 1990s has been reported in most countries.[8]Corral-Gudino L, Borao-Cengotita-Bengoa M, Del Pino-Montes J, et al. Epidemiology of Paget's disease of bone: a systematic review and meta-analysis of secular changes. Bone. 2013 Aug;55(2):347-52.
http://www.ncbi.nlm.nih.gov/pubmed/23643679?tool=bestpractice.com
PDB may be either monostotic (25%), involving most frequently the femur, or polyostotic (75%), involving most frequently the femur, pelvis, skull, tibia, or vertebrae.[1]Nance MA, Nuttall FQ, Econs MJ, et al. Heterogeneity in Paget disease of the bone. Am J Med Genet. 2000 Jun 19;92(5):303-7.
http://www.ncbi.nlm.nih.gov/pubmed/10861657?tool=bestpractice.com
[2]Hocking L, Slee F, Haslam SI, et al. Familial Paget's disease of bone: patterns of inheritance and frequency of linkage to chromosome 18q. Bone. 2000 Jun;26(6):577-80.
http://www.ncbi.nlm.nih.gov/pubmed/10831928?tool=bestpractice.com