Background. Studies from several countries suggest that the incidence of Paget’s disease of bone and the severity of newly-diagnosed cases are declining. Secular changes in clinical presentation of Paget’s disease have not been examined in Australia, which historically had the highest prevalence of Paget’s outside the United Kingdom. The aim of this study was to assess these trends in Western Australian patients.
Methods. The participants were 293 patients (61% male) with data available from the database of the Paget’s Disease Research Group of Western Australia. In regression models, we examined year of diagnosis as a predictor variable, age at diagnosis, number of bones involved and pre-treatment total plasma alkaline phosphatase (ALP) activity as outcome variables, with Sequestosome 1/p62 (SQSTM1) mutation status, family history, country of birth, smoking status and dog exposure as covariates.
Results. The year of diagnosis ranged from 1956 to 2013, and the mean age at diagnosis was 63 years (range 28 to 90). Twenty-six percent of participants reported a family history of Paget’s disease and 11% had SQSTM1 mutations. There was a significant positive relationship between year of diagnosis and age at diagnosis (p<0.0001) and significant negative relationships between year of diagnosis and both pre-treatment ALP and number of involved bones (p
Conclusions. The severity of Paget’s disease of bone at diagnosis in Australia has declined over the past 5 decades. This is likely to reflect altered exposure to one or more environmental agents involved in the pathogenesis of PDB.