Abstract

Background: Monitoring changes in the epidemiology of cystic fibrosis (CF) pathogens further improves our understanding of the potential impact of interventions. Methods: We performed a retrospective analysis using data reported to the European Cystic Fibrosis Society Patient Registry (ECFSPR) from 2011 to 2016 to determine the overall and the age specific annual prevalence and incidence of selected CF pathogens and their trends during these years. The ECFSPR collects data on three chronic infections: Pseudomonas aeruginosa (PsA), Burkholderia cepacia complex Species (BCC) and Staphylococcus aureus (SA), as well as on the occurrence of non-tuberculous mycobacteria (NTM) and Stenotrophomonas maltophilia (SM). The same analyses were performed after stratifying for different country groups, according to their gross national income (GNI). Findings: The pathogens with highest prevalence were SA and PsA, with prevalence rates, in 2016, equal to 38.27% and 29.83% respectively, followed by SM (8.14%). The pathogens with lowest prevalence were NTM (3.33%) and BCC (3.13%). The overall prevalence and incidence significantly decreased for PsA; they also decreased non-significantly for BCC, while they increased significantly for SA. The overall prevalence of NTM and SM increased significantly. Among the CF pathogens studied, PsA showed the largest changes in prevalence: it significantly decreased in all age strata, (except 0-1 years) and in all income countries groups. The prevalence and incidence of pathogens differed significantly according to GNI. For PA, BCC and SA the prevalence was significantly higher in lower income groups, while the prevalence and incidence of NTM was higher in countries with the highest income. Interpretation: The epidemiology of CF pathogens in Europe has changed over the 6-year study period; epidemiologic data differ significantly among countries with different socioeconomic status. The causes of these observations are likely multifactorial and include improvements in clinical care and infection prevention and control. Funding: None. Declaration of Interest: We declare that we have no conflicts of interest. Ethical Approval: Scientific and Steering committees of the European Cystic Fibrosis Society Patient Registry.

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