M Varenna1, F Zucchi2, C Crotti2, M Manara3, R Caporali3,4. 1. Bone Diseases Unit, Department of Rheumatology, Gaetano Pini Institute, Via Pini, 9, 20122, Milan, Italy. massimo.varenna@asst-pini-cto.it. 2. Bone Diseases Unit, Department of Rheumatology, Gaetano Pini Institute, Via Pini, 9, 20122, Milan, Italy. 3. Department of Rheumatology, Gaetano Pini Institute, Milan, Italy. 4. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
Abstract
Patients with Paget's disease of bone recruited over the last 20 years by a single centre were evaluated to find possible clinical changes. All markers of severity showed consistent downward trends. A reduced disease incidence could seemingly refer to lower sensitivity of the diagnostic tools owing to lower severity. INTRODUCTION: This study aimed to evaluate if the severity of Paget's disease of bone (PDB) is decreasing and whether a milder phenotype can have affected the results of studies on disease prevalence. METHODS: From August 2007 to August 2019, 167 patients with PDB were referred to our centre. Demographic and clinical characteristics were collected and compared with those of a sample of 224 patients enrolled in the same setting between January 2000 and July 2007. Multivariate analyses on 391 patients as a whole were performed assuming the year of presentation as explanatory variable. RESULTS: Patients of newer sample were diagnosed at a significantly older age (64.0 ± 11.3 vs 61.1 ± 11.6; p = 0.01). By comparing clinical features acknowledged as markers of disease severity, the mean number of involved bones, the proportion of skeletal involvement, and pre-treatment serum alkaline phosphatase (SAP) values all showed significant decreases (p < 0.001) in the more recent sample. Multivariate analyses confirmed these results for the latter two indices. Further markers of disease severity such as the prevalence of monostotic disease and normal SAP at diagnosis showed the same trend. The sensitivity of tools allowing incidental diagnosis in asymptomatic patients showed a reduced sensitivity: -11% for radiological assessments and -33% for SAP. CONCLUSIONS: Allowing for referral differences, our study provides information on reduced severity of PDB over the last two decades. A milder phenotype affects the age at onset and impairs the sensitivity of the diagnostic tools contributing to reduce the prevalence of PDB patients incidentally discovered.
Patients with Paget's disease of bone recruited over the last 20 years by a single centre were evaluated to find possible clinical changes. All markers of severity showed consistent downward trends. A reduced disease incidence could seemingly refer to lower sensitivity of the diagnostic tools owing to lower severity. INTRODUCTION: This study aimed to evaluate if the severity of Paget's disease of bone (PDB) is decreasing and whether a milder phenotype can have affected the results of studies on disease prevalence. METHODS: From August 2007 to August 2019, 167 patients with PDB were referred to our centre. Demographic and clinical characteristics were collected and compared with those of a sample of 224 patients enrolled in the same setting between January 2000 and July 2007. Multivariate analyses on 391 patients as a whole were performed assuming the year of presentation as explanatory variable. RESULTS:Patients of newer sample were diagnosed at a significantly older age (64.0 ± 11.3 vs 61.1 ± 11.6; p = 0.01). By comparing clinical features acknowledged as markers of disease severity, the mean number of involved bones, the proportion of skeletal involvement, and pre-treatment serum alkaline phosphatase (SAP) values all showed significant decreases (p < 0.001) in the more recent sample. Multivariate analyses confirmed these results for the latter two indices. Further markers of disease severity such as the prevalence of monostotic disease and normal SAP at diagnosis showed the same trend. The sensitivity of tools allowing incidental diagnosis in asymptomatic patients showed a reduced sensitivity: -11% for radiological assessments and -33% for SAP. CONCLUSIONS: Allowing for referral differences, our study provides information on reduced severity of PDB over the last two decades. A milder phenotype affects the age at onset and impairs the sensitivity of the diagnostic tools contributing to reduce the prevalence of PDB patients incidentally discovered.
Authors: Daniela Merlotti; Luigi Gennari; Beatrice Galli; Giuseppe Martini; Anna Calabrò; Vincenzo De Paola; Elena Ceccarelli; Paolo Nardi; Annalisa Avanzati; Ranuccio Nuti Journal: J Bone Miner Res Date: 2005-03-28 Impact factor: 6.741
Authors: Luigi Gennari; Marco Di Stefano; Daniela Merlotti; Nicola Giordano; Giuseppe Martini; Cristina Tamone; Roberto Zatteri; Roberto De Lucchi; Carlo Baldi; Angelo Vattimo; Silvia Capoccia; Luca Burroni; Simone Geraci; Vincenzo De Paola; Anna Calabrò; Annalisa Avanzati; Giancarlo Isaia; Ranuccio Nuti Journal: J Bone Miner Res Date: 2005-05-31 Impact factor: 6.741
Authors: Cameron Britton; Suzanne Brown; Lynley Ward; Sarah L Rea; Thomas Ratajczak; John P Walsh Journal: Calcif Tissue Int Date: 2017-09-07 Impact factor: 4.333