Background: The prevalence and severity of Pagets disease of bone (PDB) have fallen over recent years but it is unclear if this has impacted on disease presentation. Here we evaluated the presentation of PDB in a contemporary cohort and conducted a systematic review of MEDLINE on the mode of presentation.
Methods: The presenting features of PDB were recorded in 87 patients who had presented to a specialist clinic between 2005 and 2013. The systematic review was conducted using the MESH terms Pagets disease of bone or osteitis deformans and the keyword clinical. The abstracts of these citations (n=415) were analysed and relevant data extracted.
Results: The incidence of clinically diagnosed PDB in our region was 0.13 cases/10 000 population per year compared with an age-adjusted incidence of 0.70 cases/10 000 per year in the general population. Based on this we estimate that 18% of patients had come to medical attention. Skeletal pain was the presenting feature in 75% of cases but only 36% were thought to have pain caused by PDB. About 5% presented with a pathological fracture; 14% with bone deformity and 1% with deafness. Only 21% of patients were asymptomatic. The patients with bone pain thought to be due to PDB were treated with bisphosphonates and 58% reported an improvement but no tangible improvement was noted in deformity or deafness. The systematic review also showed that bone pain was the most common presenting feature in other case series with variable frequencies of other complications.
Conclusion: Most patients with PDB who come to clinical attention are symptomatic and about 15% have complications by the time they present. Although PDB has become less common in recent years, there remains a clinical need to develop biomarkers for early detection of PDB so that treatment can be initiated before irreversible skeletal damage has occurred.
17 - 20 May 2014
European Calcified Tissue Society