Epidemiology of Paget's Disease in Europe: The Prevalence Is Decreasing

Abstract

This study estimated changes in the age-and sex-specific prevalence of Paget's disease of bone in six European towns over a 20-year period. Declines in prevalence were observed in this disorder, occurring among both men and women.Introduction: To estimate secular changes in the age-and sex-specific prevalence of Paget's disease of bone in Europe, we conducted a second radiographic survey using identical sampling and methods in six European towns where a baseline study was performed in [1978][1979]. In addition to these towns, the survey was carried out in two Hungarian centers not included in the initial study. Materials and Methods: In each center, a sample of abdominal radiographs of people Ն55 years of age was taken from stored films within the radiology department of the principal general hospital. Radiographs showing the entire pelvis, sacrum, femoral heads, and lumbar vertebrae were studied for the period of 2000-2001. The films were evaluated by a trained observer and a consultant radiologist. Results: A total of 6935 radiographs (3512 women and 3423 men) were assessed in the eight towns. The overall age-and sex-standardized prevalence rate of Paget's disease was 0.3% with a male/female ratio of 1.5. Prevalence increased with age among men and women rising to 0.8% of men and 0.9% of women Ն85 years of age. The differences in prevalence rate among the European centers were relatively small, especially in women. There was a decline in rates between 1978/79 and 2000/01. Conclusions: These European data confirm the decrease in frequency of Paget's disease observed in Britain. These declines favor an environmental contribution to the causation of the disease that requires further research.

Genetic aspects of the Paget’s disease of bone: concerns on the introduction of DNA‐based tests in the clinical practice. Advantages and disadvantages of its application

“…According to several studies [1,2], the described time trends of PDB have revealed: (i) a decline of the frequency of PDB in recent years; however, it should also be stressed that there have been major changes in the ethnic make-up of the population in UK over the past 25 years with an influx of people from the Indian subcontinent and Asia, more in general, which could account at least in part for the described reduced prevalence. Moreover, as PDB diagnosis in the past was accidentally performed, in particular in asymptomatic or slightly symptomatic forms, when classical radiology was intended to evaluate abdominal symptoms, back pain, pelvic pain or hip pain, the advent of ultrasonography in evaluating abdominal disorders, such as gallstones and kidney stones, has supplanted the traditional radiology in the diagnosis of these diseases and consequently reduced the possibility of accidentally identifying a bone disease such as PDB.…”

“…It has been suggested that patients with SQSTM1 ⁄ p62 mutations may exhibit a more severe phenotype (earlier onset and greater skeletal extent) than patients without mutations, and truncating mutations tend to cause a more severe phenotype than missense mutations [2,8,45,55].…”

“…PDB is quite common amongst older people of Western European descent, representing the second metabolic bone disorder (the first one is osteoporosis) affecting individuals over 55 years of age [1,2]. hyperphosphatasia) [8] and the syndrome of hereditary inclusion body myopathy, PDB and frontotemporal dementia (IBMPFD) [9]] have been identified.…”

Enfermedad de Paget. Actualización en el tratamiento médico

“…Se ha descrito una agregación familiar entre un 15% y un 40% de los pacientes (8) y en estos casos la enfermedad se transmite de forma autosómica dominante con una penetrancia variable. En los últimos 15 años, varios estudios en diferentes poblaciones han observado un descenso tanto en la prevalencia como en la gravedad de la enfermedad (9,10). Se desconocen las razones de este descenso, pero podría estar en relación con los movimientos migratorios, la disminución de la consanguinidad y carga genética y con cambios en los factores ambientales.…”

Headache in patients with Paget’s Disease of Bones

“…It is still debatable in the literature if paramyxoviruses (particularly measles and canine distemper) infection in childhood is the cause of PDB and, thus, the extensive vaccination against measles led to the decline of the disease's prevalence over the last decades 2 . Furthermore, several studies indicate mutations in the SQSTM1 gene as a predisposing genetic factor in some patients 3 .…”