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Keywords:

  • osteitis deformans;
  • epidemiology;
  • paleopathology;
  • historic;
  • europe

Abstract

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Disclosures
  8. Acknowledgements
  9. References

The strong genetic component in the etiology of Paget's disease of bone (PDB), together with marked geographic variation in its prevalence, with high frequencies in British populations, has led some to suggest that the disease originated in Britain and spread around the world in recent times by the migration and admixture of British populations. This study aims to investigate this hypothesis by studying the world geographic distribution of PDB cases identified in ancient skeletons excavated from archaeological sites. The methodology is a review of PDB cases described in the literature. There were 109 cases that met modern diagnostic criteria. All came from Western Europe, 94% from England. These data support the hypothesis that PDB originated in this geographic region. © 2010 American Society for Bone and Mineral Research


Introduction

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Disclosures
  8. Acknowledgements
  9. References

Paget's disease of bone (PDB) is a condition characterised by excessive and abnormal bone remodeling and formation of bone that is structurally abnormal. The etiology of PDB remains incompletely understood. However, current consensus appears to favor the action of environmental factors, most likely viral agents, on a genetically susceptible host. The evidence for genetic predisposition is compelling.1 This, coupled with the marked geographic variation in prevalence of PDB, with high prevalences in populations of British origin, has led to the suggestion that the disease originated in Britain and was spread around the world by migration and admixture of British populations in recent times.2 The aim of this work is to investigate this suggestion using the geographic distribution of PDB cases in ancient skeletons excavated from archaeological sites.

Methods

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Disclosures
  8. Acknowledgements
  9. References

The methodology for the study is a review of the world literature on PDB in archaeological skeletons published from 1889 to the present. Much of the older literature on archaeological cases of PDB relies for diagnosis on the gross appearance of specimens. This is problematic because the pitted, thickened appearance of bones in PDB resembles that in a variety of other conditions.3 Clear radiographic views and/or microscopic demonstration of mosaic bone is needed for secure diagnosis. Only cases that met current radiographic and/or histologic diagnostic criteria4–6 and that are firmly dated to chronologic period were included in this study.

Results

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Disclosures
  8. Acknowledgements
  9. References

One-hundred and nine archaeological cases of PDB were identified. The earliest cases come from the Roman Empire (first to fourth centuries AD) (Fig. 1 and Table 1). No cases come from outside Western Europe, and 94% of cases come from England. The English cases show no particular geographic distribution; the largest numbers come from London, but this simply reflects the intensity of archaeological excavations there. There is no evidence for the Lancashire focus noted in the disease today.7, 8 However, archaeological cemetery excavations from that area have been rather few.

thumbnail image

Figure 1. Distribution of archaeological cases of PDB in Europe (no cases were found outside Europe). (Inset) Distribution of cases in England.

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Table 1. PDB Cases by Archaeological Period
 Country
PeriodEnglandGermanyFrancePortugal
Roman Empire (1–400 AD)7 11
Early Medieval (400–1066 AD)7   
Late Medieval (1066–1538 AD)5231 
Post-Medieval (1538–1850)37   
TOTAL103321

Skeletal complications reported in ancient cases of PDB include secondary osteoarthritis, bone deformity,9 pathologic fracture (Fig. 2), and osteosarcoma.10 Inconsistencies in reporting meant that the frequency of such complications could not be quantified.

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Figure 2. (A) Radiograph of part of a femur from a case of PDB in a medieval (twelfth to sixteenth century AD) skeleton from Ipswich, England. The arrow shows the location of an nonunited fracture. (B) Although nonunited, the broken ends of the fracture demonstrate remodeling, indicating that the fracture was sustained shortly before death.

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Of the 109 cases of PDB included in this study, the sex of the affected individual was reported in 72 cases: 51 were male and 21 female. The sex imbalance in favor of males is statistically significant (χ2 = 12.5, p < .001). This is concordant with results from modern populations, where a male sex bias generally (but not invariably) is reported.7, 8, 11–13

Discussion

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Disclosures
  8. Acknowledgements
  9. References

In the current data, convincing archaeological cases of PDB are restricted to Western Europe, and the overwhelming majority come from England. The lack in the literature of credible archaeological cases from other regions of the world, including the great civilizations of ancient Egypt, the Middle and Far East, and Andean South America, is noteworthy.

There are some limitations to the current study. First, figures for the total number of ancient skeletons excavated from different parts of the world are not available. In countries with developed archaeological sectors, it is routine that skeletal remains are excavated when building work or other redevelopment impinges on ancient burial grounds. In most countries, many thousands of archaeological burials have been collected and studied. Some lowland tropical regions are exceptions to this: Some countries in those regions lack developed archaeological infrastructure, and buried human skeletal remains often survive poorly in humid tropical environments. Nevertheless, most other regions of the world are well represented. Second, there inevitably will be some archaeological skeletons with PDB in museum stores that have not been published in the literature. Given the tendency, in the literature on ancient disease, toward publication of case studies of first examples of particular diseases seen in a region or country, examples are more likely to languish unpublished if they come from regions where ancient cases of PDB are numerous than where they are not. Therefore, this factor is likely to lead to an under- rather than overestimation of the concentration of cases in northwest Europe.

Despite the limitations of this study, it seems unlikely that the observed geographic pattern is simply an artifact of geographic differences in intensity of archaeological work, publication, or skeletal survival. It seems likely that it is largely a reflection of the geographic distribution of PDB in premodern times. The results are consistent with a northwest European origin for PDB. Another possibility is that a founder migrated to northwest Europe at some point in the past and disseminated PDB there rather than PDB originating there. This currently seems less likely given the dearth of evidence for PDB in archaeological skeletons elsewhere in the world, although, of course, future archaeological finds may change this picture.

PDB has existed in Western Europe for at least 2000 years. The preponderance of males usually seen in PDB today also seems to apply to ancient cases of the disease.

Disclosures

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Disclosures
  8. Acknowledgements
  9. References

The author states that has no conflicts of interest.

Acknowledgements

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Disclosures
  8. Acknowledgements
  9. References

I wish to thank Vince Griffin for drawing Fig. 1.

References

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Disclosures
  8. Acknowledgements
  9. References
  • 1
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