PDB is the second most common chronic bone-remodeling 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
Prevalence is approximately 1% in the general US population, rising to 2.32% in people ages 65 to 74 years.[5]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
There is a slight increased distribution in the northeast region compared with other regions in the US. 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-year age group).[5]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
An overall prevalence of 2% has been reported in the UK.[6]Cooper C, Schafheutle 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%.[7]Poór G, Donáth J, Fornet B, et al. Epidemiology of Paget's disease in Europe: the prevalence is decreasing. J Bone Miner 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
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