Prevalence of Paget's disease of bone in Spain

doi:10.1016/j.bone.2008.08.108

Bone

Volume 43, Issue 6, December 2008, Pages 1006-1009

https://doi.org/10.1016/j.bone.2008.08.108 Get rights and content

Abstract

Introduction

Epidemiological studies in Europe have revealed that the prevalence of Paget's disease of bone (PDB) has marked geographic variations. At present, the prevalence of PDB in Spain is unknown, limited to data from isolated towns or centers. We conducted a radiological national-based survey, to estimate the age and sex prevalence of PDB and its geographic variation within the country. In addition, we tested the patients' awareness of their disease.

Methods

Stratified samples throughout Spain of abdominal radiographs, of subjects aged ≥ 55 years, from stored consecutive digitalized films in selected hospitals were obtained, over the period of 2006–2007. Radiographs including all lumbar vertebrae, pelvis, sacrum and femoral heads were examined for the diagnosis of PDB, according to standardized criteria. Age, sex and information regarding patient's awareness of the illness were obtained from the hospital files.

Results

A total of 4528 radiographs from 13 centers were evaluated. The crude prevalence of PDB was 1% (95%CI: 0.7–1.3) in individuals older than 55, and the estimated prevalence ranged from 1.1% (95%CI: 0.8–1.4) to 1.6% (95%CI: 1.1–2.1) when a reported pelvic involvement in 60–90% of PDB patients was considered. The prevalence was slightly higher in men than in women, and significantly higher in individuals older than 75. A significant geographic variation in prevalence was observed within the country (p = 0.004). 73% of PDB patients were unaware of their illness at the time of the radiological survey.

Conclusions

Prevalence of PDB in Spain is at least 1% in individuals older than 55, with remarkable geographical variations and age related differences. Most patients were unaware of their disease.

Introduction

Paget's disease of bone (PDB) is the second most frequent metabolic bone disease, which usually occurs in elderly patients and has a strong genetic component [1]. It is known that PDB prevalence in Europe has remarkable geographic variations. The highest prevalence (3–5% in population over the age of 55) has been reported in Britain whilst the lowest indices have been found in the Scandinavian population (0.4%) [2], [3]. Also, a low prevalence of PDB in Southern Europe has been described [4]. A recent survey in Italy estimated that the overall prevalence of PDB, by radiological assessment, ranges from 0.7 to 1.5% in subjects older than 60, increasing to 2.4% when a scintigraphic survey in an over 43-year old population from Siena, was performed [5]. At present, there is little information regarding the prevalence of PDB in other Southern European countries, and reports are limited to studies providing data from local surveys [4], [6], [7], [8].

The reported decrease in frequency of PDB in the last two decades in a number of European centers and in New Zealand [6], [9], [10], and the lower number of symptomatic patients at diagnosis in the late periods of long surveys may modify the previous reported prevalences of PDB [9]. Furthermore, since this is frequently an asymptomatic condition [11], [12], it is interesting to assess whether patients are aware of their disorder in order to seek medical care.

To estimate the total and the sex and age prevalence of PDB in Spain, as well as the patients' awareness of the disorder, we conducted a radiographic survey using abdominal radiographs in a national-based study.

Section snippets

Methods

We selected 18 geographic areas of the country according to the distribution in Autonomous Communities of Spain and stratified them, taking into account their population. A total of 25 centers from these areas were selected at random using the National Hospital Catalog. The number of centers per stratum was proportional to the population aged 55 in any given stratum.

Abdominal radiographs of consecutive subjects aged ≥ 55 years were obtained from the stored digitalized films in the radiology

Results

In total, 4528 abdominal radiographs were evaluated. For reasons related to the standardized criteria for the radiological survey that required digitalized radiographs, only 13 centers could finally take part in the study from the initially 25 selected. The geographic distribution throughout the country and the number of films recruited per geographical stratum is shown in Table 1. The average number of radiographs examined per center was 350 (range 148–700). The reason for having assessed less

Discussion

The results of this national-based radiological survey contribute to the knowledge of the epidemiology of PDB, providing new information on the prevalence and the geographic distribution of this disorder in Spain. The methodology of this survey, evaluating abdominal radiographs, has been previously used in epidemiological studies in Britain, Italy, the United States and New Zealand, as well as in one study assessing six European towns [4], [5], [14], [15], [16]. When we compare our data with

Acknowledgments

This work was funded by a grant from Novartis Farmacéutica, S.A. We are most grateful to the Spanish Society of Rheumatology and the Spanish Society of Bone and Mineral Metabolism for their support, as well as to Maria Victoria Pérez Guijo for the data entry.

References (24)

CooperC. et al.
Epidemiology of Paget's disease of bone
Bone
(1999)
TiegsR.D. et al.
Long-term trends in the incidence of Paget's disease of bone
Bone
(2000)
DoyleT. et al.
Paget's disease in New Zealand: evidence for declining prevalence
Bone
(2002)
GuyerP.B.
Paget's disease of bone: the anatomical distribution
Metab. Bone Dis. Relat. Res.
(1981)
DavieM. et al.
Paget's disease of bone: a review of 889 patients
Bone
(1999)
Morales-PigaA.A. et al.
Is clinical expressiveness of Paget's disease of bone decreasing?
Bone
(2002)
LylesK.W. et al.
A clinical approach to diagnosis and management of Paget's disease of bone
J. Bone Miner. Res.
(2001)
CooperC. et al.
Update on the epidemiology of Paget's disease of bone
J. Bone Miner. Res.
(2006)
DetheridgeF.M. et al.
European distribution of Paget's disease of bone
Br. Med. J. (Clin Res Ed)
(1982)
GennariL. et al.
Prevalence of Paget's disease of bone in Italy
J. Bone Miner. Res.
(2005)

PoorG. et al.

Epidemiology of Paget's disease in Europe: the prevalence is decreasing

J. Bone Miner. Res.

(2006)

PigaA.M. et al.

Risk factors for Paget's disease: a new hypothesis

Int. J. Epidemiol.

(1988)

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Has Paget's bone disease become rare?
2019, Joint Bone Spine
Has Paget's disease become a rare bone disease?
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La maladie de Paget était traditionnellement la seconde maladie métabolique osseuse en fréquence dans les pays d’Europe occidentale. Sur les continents asiatique et africain, elle a toujours été rare. La diminution de l’incidence et de la prévalence a été constatée de façon concordante dans de nombreux travaux épidémiologiques que l’approche soit clinique (signes et complications de la maladie), radiologique (détection fortuite des formes asymptomatiques) ou biologique (dosage de marqueurs osseux). Parallèlement, la sévérité de la maladie est aussi déclinante dans la plupart des régions où elle a été évaluée. Les causes de cette raréfaction sont essentiellement environnementales : diminution de l’exposition à des agents pathogènes ou à des habitudes quotidiennes. Cette raréfaction de la maladie expose les futurs médecins à une méconnaissance par défaut de formation et les malades à un retard de prise en charge diagnostique et thérapeutique.
Paget's disease was traditionally the second most common bone metabolic disease in Western European countries. On the Asian and African continents, it has always been rare. The decrease in incidence and prevalence has been found to be consistent in many epidemiological studies with several approaches: clinical (signs and complications of the disease), radiological (fortuitous detection of asymptomatic forms) or biological (bone marker assays). At the same time, the severity of the disease is also declining in most of the regions where it has been evaluated. The causes of this scarcity are essentially environmental: decreased exposure to pathogens or daily habits. The rarefaction of the disease exposes the future doctors to a lack of knowledge by default of training and exposes the patients to a delay for diagnostic and therapeutic management.
Polymorphisms in genes implicated in base excision repair (BER) pathway are associated with susceptibility to Paget's disease of bone
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Citation Excerpt :
Currently, PDB is the second most frequent bone disorder and affects up to 3% of Caucasians over 55 years old [7]. In Spain the prevalence is 1,3% with an irregular geographic distribution leading to areas of high prevalence like the Vitigudino region (Salamanca) with a prevalence of 5,7% [8]. The most accepted etiopathogenic hypothesis to explain the origin of the PDB considers that PDB is the consequence of the synergic action of environmental and genetic factors.
Paget's disease of bone (PDB) is a chronic bone metabolic disorder. Currently, PDB is the second most frequent bone disorder. PDB is a focal disorder affecting the skeleton segmentally but the cause of which is unknown. It has been hypothesised that somatic mutations could be responsible for the mosaicism described in PDB patients. Therefore, our hypothesis is that defective response to DNA damage may lead to somatic mutations favouring an increased risk of PDB. So that we have analysed polymorphisms in DNA repair genes involved in the BER, NER and DSBR pathways in order to evaluate the role of these variants in modulating PDB risk. We found statistically significant differences in genotypic and allelic distribution for polymorphisms in genes implicated in the BER pathway. Our results showed that carrying the allele T of XRCC1 rs1799782 polymorphism and the allele G of APEX rs1130409 polymorphism increased the risk of developing PDB. These polymorphisms could cause a lower DNA repair efficiency and this might lead to local somatic mutations favouring bone metabolic alterations characteristic of PDB. This is the first report showing an association between polymorphism in genes implicated in the BER pathway with PDB.
Environmental factors associated with familial or non-familial forms of Paget's disease of bone
2018, Revue du Rhumatisme (Edition Francaise)
La mutation du gène SQSTM1 la plus fréquemment retrouvée dans la maladie osseuse de Paget (MOP), p.Pro392Leu, reproduit certaines caractéristiques phénotypiques de la MOP mais semble insuffisante pour aboutir au phénotype pagétique complet, ce qui suggère la possible implication de facteurs environnementaux dans la pathogénèse de la MOP. Nous avons mené une étude exploratoire afin d’identifier les facteurs environnementaux potentiellement associés aux formes familiales et non familiales de MOP dans la population canadienne-française.
Nous avons recherché les facteurs environnementaux à partir d’un questionnaire adressé à 176 patients pagétiques dont 86 atteints d’une forme familiale et à 147 témoins sains. Tous les participants résidaient dans la même zone géographique et dans un rayon de 120 km autour de la ville de Québec. Les associations entre les facteurs environnementaux et les formes familiales et non familiales de MOP ont été recherchées.
Dans un modèle multivarié ajusté sur la corrélation intrafamiliale, la MOP était associée au chauffage au bois durant l’enfance et/ou l’adolescence (OR = 2,10 ; IC 95 % 1,13–3,90, p = 0,02). Dans l’analyse multivariée, sans prise en compte de la corrélation intrafamiliale, la forme familiale de MOP était associée à la présence d’une mine à proximité du lieu de résidence (OR = 11,70 ; IC 95 % 2,92–46,80, p < 0,01) et à la pratique de la chasse (OR = 2,92 ; IC 95 % 1,14–7,47, p = 0,03). Le chauffage au bois durant l’enfance et/ou l’adolescence (p = 0,02) était associé aux formes familiales et non familiales.
En conclusion, la MOP était significativement associée au chauffage au bois durant l’enfance et/ou l’adolescence, qu’il s’agisse des formes familiales ou non.
Paget's disease of bone: Diagnostic and therapeutic updates
2018, Revue de Medecine Interne
La maladie osseuse de Paget (MOP) est la deuxième plus fréquente maladie osseuse métabolique après l’ostéoporose. Son étiopathogénie n’est pas encore clairement élucidée. La distribution géographique et les variations épidémiologiques suggèrent un rôle de la génétique et des facteurs d’environnement dans sa physiopathologie. La fréquence de la MOP augmente avec l’âge. Sa découverte peut être fortuite à l’occasion d’un bilan biologique ou radiographique et son diagnostic impose un bilan d’extension et d’évolutivité. Son pronostic dépend essentiellement de la survenue de complications. Ces complications peuvent être osseuses, articulaires, neurologiques, cardiovasculaires ou métaboliques. Son traitement concerne les formes symptomatiques, évolutives ou à risque de complications. Il repose actuellement sur les bisphosphonates qui ont transformé son pronostic.
Paget's disease of bone is the second most common metabolic bone disease after osteoporosis. Its pathogenesis is not yet clearly understood. Geographic distribution and epidemiological variations suggest a role of genetic and environmental factors in its pathophysiology. The frequency of the Paget's disease of bone increases with age. Its discovery can be fortuitous. Prognosis mainly depends on the occurrence of complications involving bones and joints, neurological, cardiovascular or metabolic systems. Treatment of symptomatic forms currently relies on bisphosphonates that have transformed its prognosis.
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View all citing articles on Scopus

^☆: The study was supported by a grant from Novartis Pharma, España. The authors state that they have no conflicts of interest.

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Prevalence of Paget's disease of bone in Spain☆

Abstract

Introduction

Methods

Results

Conclusions

Introduction

Section snippets

Methods

Results

Discussion

Acknowledgments

Bone

Bone

Bone

Metab. Bone Dis. Relat. Res.

Bone

Bone

A clinical approach to diagnosis and management of Paget's disease of bone

J. Bone Miner. Res.

Update on the epidemiology of Paget's disease of bone

J. Bone Miner. Res.

European distribution of Paget's disease of bone

Br. Med. J. (Clin Res Ed)

Prevalence of Paget's disease of bone in Italy

J. Bone Miner. Res.

Epidemiology of Paget's disease in Europe: the prevalence is decreasing

J. Bone Miner. Res.

Risk factors for Paget's disease: a new hypothesis

Int. J. Epidemiol.